Lymphoma rib cage pain. Primary Rib Non-Hodgkin’s Lymphoma: A Rare Case of Bone Lymphoma Mimicking Male Breast Cancer

10.09.202314.06.2023 by alexxlab

What are the key features of primary rib non-Hodgkin’s lymphoma. How does it differ from typical lymphoma presentations. What diagnostic challenges does it pose. How is primary bone lymphoma treated. What is the prognosis for patients with this rare condition.

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Understanding Primary Lymphoma of Bone: A Rare Entity

Primary lymphoma of bone is an uncommon condition that poses unique diagnostic and treatment challenges. Unlike typical lymphomas that arise in lymph nodes, this rare form originates directly in bone tissue. While it can affect any bone in the body, occurrence in the ribs is particularly unusual.

Key characteristics of primary bone lymphoma include:

Direct origin in bone tissue rather than lymph nodes
Often presents with localized bone pain and swelling
May be mistaken for other bone tumors or metastases
Typically non-Hodgkin’s lymphoma, B-cell type
Requires specialized imaging and biopsy for accurate diagnosis

Clinical Presentation: When Lymphoma Masquerades as Breast Cancer

The case report describes a 60-year-old male patient who presented with right chest and back pain. This symptom alone is non-specific and could indicate various conditions. However, the location and nature of the pain led to an initial suspicion of male breast cancer, highlighting the deceptive nature of this rare lymphoma.

Why was breast cancer initially suspected? Several factors contributed:

Pain localized to the chest wall
Presence of a palpable mass
Rarity of primary rib lymphoma
Higher incidence of breast cancer compared to bone lymphoma

This case underscores the importance of maintaining a broad differential diagnosis when evaluating chest wall masses, especially in older adults.

Diagnostic Journey: Unraveling the Mystery

Diagnosing primary rib lymphoma requires a systematic approach and advanced imaging techniques. In this case, the diagnostic process involved:

Initial chest X-ray
Computed tomography (CT) scan
Ultrasonically-guided needle biopsy
Histopathological examination

The CT scan revealed a critical finding: osteolysis (bone destruction) in the sixth right rib, accompanied by a surrounding soft tissue mass. This imaging characteristic is often seen in aggressive bone tumors but can be found in both primary bone lymphomas and metastatic lesions.

How did the biopsy confirm the diagnosis? The histologic examination showed:

Dense proliferation of atypical cells
Medium-sized cells with prominent nucleoli
Irregular nuclear borders

These features led to the definitive diagnosis of diffuse, medium-sized non-Hodgkin’s lymphoma, B-cell type, originating in the rib.

Treatment Approach: Balancing Local Control and Systemic Therapy

Managing primary bone lymphoma presents unique challenges due to its localized nature within a systemic disease. In this case, the treatment strategy included:

En bloc surgical resection of the tumor and affected chest wall
Postoperative radiation therapy
Systemic chemotherapy

Why was surgery chosen as the initial treatment? Several factors influenced this decision:

Potential for complete tumor removal
Minimal impact on respiratory function
Opportunity for better local control compared to radiation alone
Ability to obtain a definitive diagnosis and staging information

The surgical specimen revealed a tumor measuring 7.5 x 4.8 x 3.0 cm, confirming the extensive nature of the disease. Unfortunately, despite aggressive multimodal therapy, the patient experienced disease progression.

Disease Progression and Prognosis: A Challenging Outcome

The case report highlights the aggressive nature of this rare lymphoma, with rapid disease progression despite treatment. Key events in the patient’s clinical course included:

Local recurrence at the surgical site
Metastatic lesions in the stomach
Involvement of para-aortic abdominal lymph nodes
Disease progression occurring just 7 months after initial symptoms
Patient succumbing to the disease 10 months after symptom onset

This unfortunate outcome raises important questions about the optimal management of primary bone lymphomas. While some literature suggests that surgical resection can yield good prognoses in select cases, this patient’s experience underscores the need for more effective treatment strategies.

Lessons Learned: Improving Diagnosis and Management

This case report offers valuable insights for clinicians encountering unusual presentations of lymphoma or bone tumors. Key takeaways include:

Maintain a high index of suspicion for rare entities like primary bone lymphoma
Utilize a multimodal diagnostic approach, including advanced imaging and biopsy
Consider the pros and cons of surgical intervention in primary bone lymphomas
Recognize the potential for rapid disease progression despite aggressive treatment
Emphasize the need for ongoing research into more effective therapies

Future Directions: Advancing Treatment for Primary Bone Lymphomas

The challenges presented by this case highlight several areas for future research and clinical advancement:

Development of more targeted therapies for bone-originating lymphomas
Investigation of novel immunotherapies that may offer better systemic control
Optimization of combined modality treatments (surgery, radiation, and chemotherapy)
Identification of prognostic markers to guide treatment intensity
Establishment of standardized treatment protocols for this rare entity

As our understanding of primary bone lymphomas grows, there is hope for improved outcomes in patients facing this challenging diagnosis.

Differential Diagnosis: When Bone Lesions Aren’t What They Seem

The case of primary rib lymphoma masquerading as male breast cancer serves as a reminder of the importance of thorough differential diagnosis in oncology. When evaluating bone lesions, particularly in the chest wall, clinicians must consider a range of possibilities:

Primary bone tumors (e.g., osteosarcoma, chondrosarcoma)
Metastatic lesions from distant primary cancers
Hematologic malignancies (lymphomas, multiple myeloma)
Benign bone tumors or cysts
Infectious processes (osteomyelitis)
Metabolic bone diseases

How can clinicians differentiate between these possibilities? Key steps in the diagnostic process include:

Thorough patient history and physical examination
Comprehensive laboratory testing, including tumor markers
Advanced imaging studies (X-ray, CT, MRI, PET-CT)
Image-guided biopsy for histopathological analysis
Immunohistochemistry and molecular testing when appropriate

By following a systematic approach, clinicians can avoid misdiagnosis and ensure patients receive the most appropriate treatment for their specific condition.

The Role of Imaging in Diagnosis and Staging

Imaging plays a crucial role in the diagnosis and management of primary bone lymphomas. Each modality offers unique insights:

X-ray: Provides initial assessment of bone integrity and lesion characteristics
CT: Offers detailed visualization of bone destruction and soft tissue involvement
MRI: Excels at demonstrating bone marrow infiltration and soft tissue extension
PET-CT: Valuable for assessing metabolic activity and staging the disease

In the case of primary rib lymphoma, CT imaging revealed the classic finding of osteolysis with an associated soft tissue mass. This appearance, while suggestive of an aggressive process, is not specific to lymphoma and requires correlation with biopsy results.

Histopathological Features: Clues to Diagnosis

The definitive diagnosis of primary bone lymphoma relies heavily on histopathological examination. Key features observed in this case included:

Dense proliferation of atypical lymphoid cells
Medium-sized cells with prominent nucleoli
Irregular nuclear borders
Diffuse growth pattern

Additional immunohistochemical staining would typically be performed to confirm the B-cell origin and further characterize the lymphoma subtype. This information is crucial for guiding treatment decisions and predicting prognosis.

Treatment Challenges: Balancing Local and Systemic Approaches

Managing primary bone lymphomas requires a delicate balance between addressing the localized bone lesion and treating the underlying systemic disease. The treatment approach in this case involved:

Surgical resection for local control and definitive diagnosis
Radiation therapy to the tumor bed
Systemic chemotherapy to address potential microscopic disease

Why might this approach have been insufficient? Several factors could contribute to treatment failure:

Aggressive biology of the specific lymphoma subtype
Potential for microscopic disease spread prior to diagnosis
Limitations of conventional chemotherapy in penetrating bone
Individual patient factors affecting treatment response

The rapid disease progression in this case underscores the need for more effective treatment strategies, particularly for aggressive subtypes of primary bone lymphoma.

Prognostic Factors in Primary Bone Lymphoma

While the prognosis for primary bone lymphoma can vary widely, several factors have been associated with outcomes:

Age at diagnosis
Lymphoma subtype and grade
Extent of bone involvement (single vs. multiple sites)
Presence of soft tissue extension
Elevated lactate dehydrogenase (LDH) levels
Response to initial therapy

In the case presented, the rapid disease progression suggests an particularly aggressive biology. However, it’s important to note that outcomes can vary significantly between patients, and some individuals with primary bone lymphoma achieve long-term remission with appropriate treatment.

The Importance of Multidisciplinary Care

Managing rare and complex cases like primary rib lymphoma requires a coordinated effort from multiple specialties. A typical multidisciplinary team might include:

Hematologist-oncologists
Radiation oncologists
Orthopedic or thoracic surgeons
Radiologists
Pathologists
Pain management specialists
Oncology nurses and supportive care providers

By bringing together diverse expertise, multidisciplinary teams can develop comprehensive treatment plans tailored to each patient’s unique circumstances. This approach is particularly crucial in rare diseases where standardized treatment protocols may be lacking.

Emerging Therapies: Hope for Improved Outcomes

While the case presented had a poor outcome, ongoing research offers hope for improved treatments in primary bone lymphomas. Some promising areas of investigation include:

Novel targeted therapies (e.g., BTK inhibitors, BCL-2 inhibitors)
Immunotherapies, including CAR T-cell therapy
Radiolabeled antibodies for targeted radiation delivery
Bone-seeking chemotherapy agents
Combination approaches leveraging synergistic treatment modalities

As our understanding of the biology underlying primary bone lymphomas grows, there is potential for developing more effective and less toxic treatment strategies. Continued research and clinical trials are essential to advancing care for patients with this rare and challenging disease.

Primary lymphoma of bone originating in a rib

Case Reports

. 2000 Mar;48(3):180-3.

doi: 10.1007/BF03218117.

N Nishiyama¹, S Nakatani, K Inoue, T Katoh, H Kinoshita

Affiliations

Affiliation

¹ Department of Surgery, Kita Citizen’s Hospital of Osaka City, Japan.

PMID:
10793497
DOI:
10.1007/BF03218117

Case Reports

N Nishiyama et al.

Jpn J Thorac Cardiovasc Surg.

2000 Mar.

. 2000 Mar;48(3):180-3.

doi: 10. 1007/BF03218117.

Authors

N Nishiyama¹, S Nakatani, K Inoue, T Katoh, H Kinoshita

Affiliation

¹ Department of Surgery, Kita Citizen’s Hospital of Osaka City, Japan.

PMID:
10793497
DOI:
10.1007/BF03218117

Abstract

Malignant lymphoma originating in the bone is rare and is now recognized as being an independent clinicopathologic entity known as primary lymphoma of bone. A 60-year-old man complaining of right chest and back pain consulted our hospital for further examination. Chest X-ray and computed tomogram revealed osteolysis and a surrounding soft tissue mass in the sixth right rib. An ultrasonically-guided needle biopsy of the tumor was performed, and histologic examination indicated the dense proliferation of similar-sized atypical cells with nucleoli and an irregular nuclear border. A diagnosis of diffuse, medium-sized non-Hodgkin’s lymphoma, B-cell type was made. En block resection of the tumor and chest wall was performed. Macroscopically, the tumor measured 7.5 x 4.8 x 3.0 cm in diameter, and the histologic findings were similar to those of the preoperative needle biopsy. Unfortunately, postoperative treatment with radiation therapy and chemotherapy was ultimately unsuccessful, and a local recurrence and metastatic lesions appeared in the stomach and para-aortic abdominal lymph nodes 7 months after the first symptom appeared. The patient died 3 months later. Surgery was chosen as the initial therapy as it was considered that a rib resection would not result in serious respiratory compromise and the complete resection of the tumor would be superior to radiation therapy for local control. Some authors have reported that the surgical resection of a primary lymphoma of the bone originating in a rib can yield a good prognosis. However, it is a systemic disease and a more effective therapeutic strategy should be developed.

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World J Nucl Med. 2022 Jul 19;21(2):169-172. doi: 10.1055/s-0042-1750344. eCollection 2022 Jun.
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PMID: 35865162
Free PMC article.
Cervical Radiculopathy as the Inaugural Manifestation of Non-Hodgkin’s Lymphoma of Rib.
Puranik M, Khandelwal K, Puranik S, Saxena P, Bapat P, Khandelwal G.
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Ann Indian Acad Neurol. 2021 Nov-Dec;24(6):975-977. doi: 10.4103/aian.AIAN_5_21. Epub 2021 Apr 21.
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No abstract available.

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Publication types

MeSH terms

Unusual primary osseous Hodgkin lymphoma in rib with associated soft tissue mass: a case report and review of literature | Diagnostic Pathology

Case Report
Open Access
Published: 08 June 2012

Yang Li¹,
Xiao-bing Wang²,
Xiao-ying Tian³,
Bin Li¹ &
…
Zhi Li¹

Diagnostic Pathology
volume 7, Article number: 64 (2012)
Cite this article

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Abstract

Hodgkin lymphoma (HL) typically presents as nodal lesion and may involve extranodal sites during the progression of the disease. Primary osseous HL without any lymph node association is extremely rare and only a few such cases have been described in the literature. We present a case of unusual primary HL in rib occurring in a middle-aged female patient. Computed tomography (CT) scan revealed an osteolytic lesion was located at the right second rib and was associated with a large soft tissue mass. There was no regional lymph node involvement. CT scan of neck and abdomen was performed and showed no pathologic findings, particularly no lymphadenopathy and organomegaly could be observed. Histologically, typical binucleated Reed-Sternberg (RS) cells and lacunar cells were scattered in the background of reactive inflammation with infiltration of lymphocytes, histiocytes and eosinophilic granulocytes. By immunohistochemistry, RS cells and lacunar cells were positive for CD15 and CD30 with typical membrane and paranuclear dot-like staining pattern. However, these cells were negative for Epstein-Barr virus detection by in situ hybridization. A diagnosis of primary osseous HL was made. The patient received systemic chemotherapy and local radiotherapy, and was on regular follow-up for 24 months. There was no sign of recurrence of tumor and lymph node or bone marrow involvement. Because there is a possibility of secondary bone involvement by systemic HL, strict histological analysis and thorough radiographic examination are suggested to be necessary for accurately diagnosing this tumor when it presents as a solitary bone lesion.

Virtual slides

The virtual slide(s) for this article can be found here:

http://www.diagnosticpathology.diagnomx.eu/vs/2846916171507084

Background

Hodgkin lymphoma (HL) most commonly occurs in cervical lymph nodes, and the extranodal forms are rare accounting for less than 1% [1–3]. Typically, HL is a systemic disease in which 10% to 20% of cases show involvement of the bone during the progression of the disease [4–6]. However, primary osseous HL without any lymph node association is extremely rare and so far only a few such cases with immunohistochemical and/or molecular confirmation have been described in the literature, with none of these cases being from China [7–20]. According to Ann Arbor classification, primary extranodal HL is considered at stage I, but systemic HL with secondary bone involvement corresponds to stage IV of the disease. Therefore, it is indeed a challenge for clinicians to make a correct diagnosis when the HL presents as a solitary bone mass because inaccurate diagnosis may lead to delays in clinical treatment. Herein we describe an additional case of primary osseous HL arising in the rib in a middle-aged female patient without any lymphatic manifestation.

Case presentation

Clinical presentation and management

A 38-year-old Chinese female presented with pain at the right upper side of the chest and adjacent soft tissue swelling for 3 months. A week before admission to our hospital, she was suffering from a gradually severe pain at the chest and fever. As a result, the patient was referred to our hospital for examination and treatment. Physical examination showed the patient had a mild soft tissues edema at her right upper lateral chest wall and severe pain was elicited upon chest pressure. The patient had a low grade fever of 37.4°C. There was no weight loss and no palpable lymphadenopathy or organomegaly. The laboratory results, including blood count, differential, liver and renal function, were within the normal range. A computed tomography (CT) scan of the chest revealed a single osteolytic lesion in the right second rib with associated soft tissue mass, measuring 6.0 × 5.0 × 5.0 cm in size. The most of rib was observed to be destroyed and the involvement of adjacent pleura and right upper lobe of lung were also noted (Figure 1). The lesion showed moderate enhancement after gadolinium injection. There was no enlarged lymph node found in mediastinum and thoracic cavity. A CT scan of neck and abdomen showed no pathologic findings, particularly no lymphadenopathy could be observed. A CT guided needle biopsy was performed initially, but histopathological examination showed pieces of fibrosis with infiltration of inflammatory cells. Therefore, a surgical biopsy followed by a microscopic examination was performed. After diagnosis, the patient underwent polychemotherapy according to a modified COPP protocol (bleomycin, etoposide, adriamycin, cyclophosphamide, vincristine, oncovin, procarbazine, prednisone) for five cycles before initiation of involved field radiotherapy. The lesion was regressed, and the patient was on regular follow-up for 24 months after radiotherapy. The bone marrow examination was performed at 6 months after radiotherapy, there was no abnormality found. During the period of following-up, there was no sign of recurrence of tumor and lymph node enlargement.

Figure 1

Radiographic examination of the lesion. (a) Osteolytic lesion of the right second rib with associated soft tissue mass appeared to have a distinct border to the lung in periphery in computed tomography scan (white arrow). (b) The osseous destruction of rib showed adjacent soft tissue extensions with moderate gadolinium enhancement but no involvement of regional lymph node was observed.

Full size image

Material and methods

The surgical biopsy was routinely fixed in 10% neutral buffered formalin. The tissues were embedded in paraffin. Four micrometer-thick sections were stained with H&E. Immunohistochemical analyses were performed using the ChemMate Envision/HRP Kit (Dako, Glostrup, Denmark). The antibodies used in this study included a broad panel of antibodies against CD1a, CD3, CD4, CD8, CD15, CD20, CD30, CD38, CD43, CD45,CD56, CD79a, ALK, Pax-5, BOB-1, OCT-2, S-100 protein, pan-cytokeratin and epithelial membrane antigen (EMA). Epstein-Barr virus (EBV) infection was also assessed by in situ hybridization for EBERs according to the manufacturer’s instruction (Dako, Glostrup, Denmark). The antibodies were obtained from Dako Cytomation, Santa Cruz Biotechnology (Santa Cruz, CA, USA) and Novocastra laboratories LTD (Hong Kong, China).

Results and discussion

Under microscopic examination, the lesions were characterized by a fibrosis developing in bone marrow spaces, which was infiltrated with diffuse lymphocytes, plasma cells, histiocytes, and eosinophilic and neutrophilic granulocytes. Typical morphology of Reed-Sternberg (RS) cells, with large cytoplasms and prominent eosinophilic nucleoli, were scattered in the background of reactive inflammation. Lacunar cells, mummified cells and small foci of acidophilic necrosis were also found in the tissue. The immunohistochemistry staining of the RS population confirmed the expression of PAX-5, CD30 and CD15. However, these cells did not express CD1a, CD3, CD4, CD8, CD20, CD38, CD43, CD45, CD56, CD79a, ALK, BOB-1, OCT-2, S-100 protein, pan-cytokeratin and EMA (Figure 2). Epstein-Barr virus (EBV) status was negative. Taken together, these morphological and immunological data were consistent with a classical Hodgkin’s lymphoma, mixed cellularity, according to the WHO diagnostic criteria [1].

Figure 2

Photomicrographs of the osseous lesion. (a) Scattered lacunar cells were observed in the inflammatory background with numerous lymphocytes, histiocytes, and eosinophilic granulocytes. (b) Typical morphology of Reed-Sternberg cells, with large cytoplasms and prominent eosinophilic nucleoli were also found in the lesion (white arrow). The immunohistochemical staining of the RS cells confirmed the expression of CD30 (c) and CD15 (d) with typical membrane and paranuclear dot-like staining pattern (a-b, HE staining with original magnification × 400; c-d, immunohistochemical staining with original magnification × 400).

Full size image

Primary classical Hodgkin lymphomas (HLs) are well established lymphoproliferative neoplasms of the lymph nodes with bimodal age curve. The extranodal HLs are rare and account for less than 1%. The most common site of extranodal HLs is gastrointestinal tract, followed by the pulmonary system, thyroid, skin, genitourinary system, and central nervous system. Although osseous involvement could be observed in the late stages of HLs, it is extremely rare for patients to present with primary HLs of the bone. To our knowledge, so far primary osseous HL has been reported in approximately 30 patients in the literature. Some of the cases have been associated with extra-osseous involvement. Primary osseous HL confined to the bone is so rare that approximately 19 cases have been reported worldwide to date, only 12 patients with primary solitary osseous HL [7–14] and 7 patients with primary multifocal osseous HL disease have been reported without lymphatic manifestations [15–20]. Moreover, primary osseous HL presenting as a bone lesion with an associated soft tissue mass is quite unusual [10, 21–23]. Histologically, mixed cellularity and nodular sclerosis variant of classical HL are most common subtype of primary osseous HL in literature. In clinical, four types of osseous involvement by HL may be presented: (1) primary osseous HL with solitary lesion; (2) primary osseous HL with multifocal lesion; (3) HL simultaneously in osseous and non-osseous sites; and (4) recurrence of HL at osseous sites.

Since cases of primary osseous HL are so rare, the diagnosis should be only made by strict histological and clinical manifestation. According to the WHO criteria, a diagnosis of primary bone lymphoma must be (1) a single skeletal tumor, with or without regional lymph node involvement, and (2) multiple bone lesions without visceral or lymph node involvement [24]. Many previously described cases of primary extranodal HL appeared to have concurrent and/or subsequent nodal involvement. They are factually secondary cases in a context of systemic HL. The word “primary” should be used cautiously. In the present case, we found: (1) no superficial lymphadenopathy presented at the time of diagnosis; (2) chest, neck and abdomen radiological studies showed no involvement of lymph nodes in mediastinum, thoracic and abdominal cavity; (3) the complete blood count and differential are within the normal limits; (4) the lesion was predominant in bone with associated soft tissue mass, without positive adjacent lymph nodes; (5) live, spleen and bone marrow were free of disease at the time of diagnosis and the period of following-up; (6) the RS cells in tumor demonstrated a typical immunohistochemical profile with CD30, CD15 and PAX-5 positive. CD45, CD20 and other T-cell lineage markers were negative. These results prove the present case to be of primary osseous origin. The recent studies suggest that 2-[18 F]-fluoro-2-deoxy-d-glucose positron emission tomography/computed tomography (18 F-FDG-PET/CT) enables systemic HL with secondary bone invasion to be distinguished from primitive osseous HL. This technique is highly specific in demonstrating the isolated osseous localization of the tumor and should be recommended in all patients with putative osseous lymphoma [25].

Regarding the cellular origin of RS cells in HLs, especially for those in extranodal lesions, it remains to be determined whether RS cells are clonal B cells that have lost their B cell phenotype due to destructive somatic mutation [26]. Although recent investigations have defined a characteristic profile of recurrent copy number gains and losses in classical HL, including gains of chromosomes 2p, 9p, 16p, and 17q and losses of 13q, 6q, and 11q, cytogenetic and molecular studies of HL are limited. Several recurrent genetic lesions have been identified in RS cells, and most of them affect members of the JAK/STAT or NF-κB signaling pathways [27]. It is likely that EBV plays a role in reprogramming and survival through dysregulation of several signaling networks and transcription factors, including inactivating mutations in the TNFAIP3 tumor suppressor gene and encoding a negative regulator of NF-κB activity [28]. The RS cell furthers its own survival by attracting a supportive microenvironment of immune and stromal cells, and suppressing local immune responsiveness. Although many questions remain unanswered, a better understanding of cellular interaction may help to clarify the pathogenesis of HL and promote novel approaches for targeting the therapy of this malignant tumor.

Osseous HLs usually show osteolysis in radiological examination, it is often misdiagnosed as osteomyelitis [10, 16, 29]. The present case exhibited either osteolytic lesion with soft tissue mass or inflammatory background with eosinophilic granulocytes infiltration, which might sometimes cause diagnostic confusion with primary bone sarcoma, eosinophilic granuloma and non-Hodgkin lymphoma of bone. However, osteomyelitis and primary bone sarcoma lack the specific RS cell with immunoreactivity for CD30 and CD15. Eosinophilic granuloma of bone shows clonal neoplastic proliferation of Langerhans cells that express CD1a, S-100 and Langerin. The incidence of non-Hodgkin lymphoma of bone is higher than that of primary osseous HL. The most common subtype of non-Hodgkin lymphoma in bone is diffuse large B-cell lymphoma. Immunohistochemically, most of non-Hodgkin lymphomas of bone are positive for CD45 and B-cell or T-cell lineage markers with or without CD30 expression. Co-expression of CD15 and CD30, but lacking CD45 expression of tumor cells may be helpful to confirm the diagnosis of osseous HL. Although the detection of EBV-encoded RNA (EBER) or LMP1 is indicative of classical HL, EBV is found in only a proportion of cases, particularly in mixed cellularity and lymphocyte depleted classical HL with the highest frequency of 75% [1]. By contraries, some subtypes of non-Hodgkin lymphoma, including extranodal NK/T cell lymphoma and EBV positive diffuse large B-cell lymphoma, show EBV infection in tumor cells. The present case showed EBV negative by in situ hybridization although it presented typical histological and immunohistochemical profile of classical HL. Therefore, detection of EBV-encoded products in tumor is not a definitive point to confirm the diagnosis of HL. In addition, classical HL has to be distinguished from anaplastic large cell lymphoma (ALCL) in such an unusual extranodal location because both tumors show CD30 immunoreactivity with typical membrane and paranuclear dot-like staining pattern [30]. However, differential diagnosis between HL and ALCL can be made by expression of anaplastic lymphoma kinase (ALK), EMA and CD45 which are typical of ALCL, ALK-positive, while co-expression of CD30 and PAX5 is very helpful in differentiating HL from ALCL, ALK-negative.

Conclusion

In conclusion, only a few cases of primary osseous HL have been reported in the literature. Our additive case is also presented for its rarity of site. It is the first case of primary osseous HL occurring in Chinese. The diagnosis of primary osseous HL is difficult and should be made cautiously. Besides confirmation by strict histopathological and immunohistochemical analysis, radiographic examination is helpful for demonstrating potential extra-osseous lesion to distinguish systemic HL with secondary bone invasion from primary osseous HL. In clinical, an immediate diagnosis at the early stage of disease and timely treatment with systemic chemotherapy and local radiotherapy are required because the patients with primary osseous HL appear to have a good long-term prognosis when the lesion is restricted in bone without systemic dissemination.

Consent

Written informed consent was obtained from the patient for publication of this case report and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

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Department of Pathology, The First Affiliated Hospital, Sun Yat-sen University, 58, Zhongshan Road II, Guangzhou, 510080, China
Yang Li, Bin Li & Zhi Li
Department of Pathology, Guangdong General Hospital, 106, Zhongshan Road II, Guangzhou, 510080, China
Xiao-bing Wang
School of Chinese Medicine, Hong Kong Baptist University, 7, Baptist University Road, Kowloon Tong, Hong Kong
Xiao-ying Tian

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YL and XB W made contributions to acquisition of clinical data, and analysis of the histological features by H&E staining they are joint first co-authors and made an equal contribution to this work. XYT drafted the manuscript. ZL revised manuscript critically for important intellectual content and had given final approval of the version to be published. BL carried out the immunoassays. All authors read and approved the final manuscript.

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symptoms and signs, types, causes, diagnosis and treatment of blood cancer

Article content:

General data
Causes
Blood cancers
Manifestations of different forms of the disease
Diagnostics
Features of treatment
Forecasts

General information

The main symptoms of blood cancer arise from mutations in stem cells located in the bone marrow or the lymphatic system. As a result, cells divide uncontrollably and have an abnormal structure, spread throughout the body, disrupting the functioning of organs and tissues. Source:
Taylor J, Xiao W, Abdel-Wahab O
Diagnosis and classification of hematologic malignancies on the basis of genetics //
Blood. 2017 Jul 27;130(4):410-423. doi: 10.1182/blood-2017-02-734541. Epub 2017 Jun 9 .

These pathologies are divided into three large groups:

Leukemia – systemic lesions of hematopoiesis and peripheral blood cells. They do not have a specific localization of the tumor focus.
Lymphomas – lesions of the lymphatic system, in which there is one or more pathological foci where cancerous lymphocyte cells are found.
Paraproteinemic hemoblastoses .

Leukemias involve the bone marrow in the process, which in cancer produces immature hematopoietic cells – blasts. Lymphomas affect the lymph nodes and some of the internal organs.

For a long time, pathology can be hidden, and the symptoms of hemoblastoses are similar to each other, so cytogenetic analyzes play a decisive role in diagnosis.

It is possible to completely cure hemoblastoses, including bone marrow transplantation, but this type of cancer is characterized by relapses, the return of all symptoms of the disease due to repeated damage to hematopoietic cells.

Causes of blood tumors

To date, there is no specific established cause for the development of blood cancer. But there are certain risk factors that significantly increase the likelihood of their occurrence. Among the possible influences provoking mutations in hematopoietic cells, there are:

various types of ionizing radiation;
presence of hereditary pathologies;
exposure to chemical carcinogens, including certain types of drugs;
exposure to viruses (Epstein-Barr or T-lymphotropic virus generations) Source:
Godley LA, Shimamura A
Genetic predisposition to hematologic malignancies: management and surveillance //
Blood. 2017 Jul 27;130(4):424-432. doi: 10.1182/blood-2017-02-735290. Epub 2017 Jun 9 .

Types of blood cancer

In addition to the division into three large types – leukemia, paraproteinemic hemoblastoses and lymphomas, within each group there is a division into types of tumor processes.

Lymphomas are divided into:

Hodgkin’s (they are also lymphogranulomatosis), which have several subtypes depending on the absence or presence of special cells in the affected tissues – Sternberg-Reed.
Non-Hodgkins . According to the degree of malignancy, they can be low-, medium- or high-malignant. Source:
G.S. Tumyan
Malignant lymphomas //
Clinical oncohematology. Fundamental research and clinical practice, 2009, p.92-97.

Leukemias are divided into two types:

Acute , in which the lesion affects blasts or immature hematopoietic cells.
Chronic , involves mature cells at different stages of formation. For chronic, the appearance of blast crises is typical, when the clinic resembles an acute leukemic process.

In the group of acute leukemias, according to the type of affected cells, there is a division into:

Myeloblastic forms of which should normally form platelets, erythrocytes or leukocytes.
Lymphoblastic forms . Lymphocyte precursors of T- and B-forms are affected. Source:
O.Yu. Baranova
Acute leukemia //
Clinical oncohematology. Basic Research and Clinical Practice, 2012.

In chronic leukemia there is a division into forms:

Lymphocytic leukemia , in which B-lymphocytes are affected and their mutant forms are formed.
Myelocytic leukemia in which the granulocytic series of leukocytes suffers.

In paraproteinemic hemoblastoses, abnormal, monoclonal immunoglobulins, abnormal proteins produced by mutated cells, accumulate in the blood. This type of blood cancer includes:

Myeloma with damage to the generation of lymphocytes, anemia, damage to bone tissue and excretion of paraprotein in the urine, damage to the kidneys.
Waldenström’s macroglobulinemia is a cancer that leads to a sharp change in blood viscosity due to an abnormal amount of protein and damage to organs, including vital ones.
Heavy chain disease with accumulation in the blood of large protein molecules with different chains (usually alpha or gamma), less often delta or mu forms. Possible damage to different parts of the digestive tube, severe baldness with cachexia, fever, lesions of the lymph nodes.

Manifestations of different types of cancer

Signs of hemoblastosis largely depend on the stage of the disease. In cancer, several stages are distinguished with key characteristics:

Initial – malignant cells have just formed, they divide and accumulate, there are no external symptoms and manifestations.
Expanded – typical symptoms of a particular type of blood cancer occur, changes in the blood are the most characteristic.
Terminal – drugs stop helping, the process of normal hematopoiesis is completely suppressed, only cancer cells are formed.
Remission stage (complete or incomplete possible) with restoration of normal parameters in the peripheral blood and in the bone marrow.
Relapse stage – the return of symptoms of the disease with the appearance of cancer cells after a certain period of remission.

The main manifestations of blood cancer are typical for advanced and terminal stages. Although each hemoblastosis may have its own specific symptoms. In general, the following manifestations are typical for them:

frequent colds with complications due to a decrease in the immune functions of the blood;
anemia that does not respond to therapy;
soreness of bones and large joints;
constant weakness, fatigue, pallor.

Leukemia will typically present with anemia and weakness, attacks of dizziness, bruising and hemorrhage in the whites of the eyes, bleeding gums, severe pain in the bones, enlarged liver with spleen.

Lymphoma is also characterized by lymphadenopathy (permanent and pronounced enlargement of the lymph nodes), night sweats, fever due to weight loss, skin itching.

Myeloid disease is characterized by bone fractures, increased thirst with large amounts of urine, kidney damage, anemia and frequent colds, bleeding from wounds and injection sites.

Diagnosis of blood cancer

Diagnosis is based on laboratory tests to detect cancer cells in peripheral blood and bone marrow aspirates.

For acute and chronic leukemia, it is necessary to perform a detailed blood test, study of bone marrow taken during puncture and trepanobiopsy. Supplement the diagnosis of ultrasound of the abdominal cavity, X-ray of the bones of the chest and skull, CT or MRI of the body with the study of lymph nodes. Source:
A.A. Mayboroda
On the issue of diagnosing oncological diseases //
Siberian Medical Journal, 2015, No. 2, pp. 128-134.

For lymphoma, the basis of diagnosis is the study of punctate lymph nodes, the presence of specific cells in the material. The examination is supplemented by X-ray, ultrasound and MRI of the lymph nodes.

For paraproteinemic hemoblastoses, blood and urine tests are important, including detection of specific proteins, MRI or CT, and ultrasound of the abdomen and kidneys.

Features of the treatment of blood cancer

In the treatment of blood cancer, various methods are used that have a fairly high efficiency. Shown:

Surgical treatment for certain types of cancer.
Chemotherapy.
Bone marrow stem cell transplant.
Radiation methods of treatment.
Palliative care (blood, erythrocyte, platelet transfusions).

Chemotherapy is administered with drugs that inhibit the growth of abnormal cells, in the face of massive cell death, a blood transfusion is performed to make up for the lack of one’s own cells. Bone marrow transplantation from a compatible donor helps to restore the synthesis of normal blood cells, normalizes performance. Irradiation is indicated before bone marrow transplantation to completely suppress the activity of cancer cells. Operations are used to remove the affected spleen or lymph nodes.

Treatment of hemoblastoses is accompanied by side effects, so it is necessary to carry out supportive, symptomatic therapy and a long course of rehabilitation.

Predictions

In many respects, the prognosis depends on the type of blood cancer and its stage, the existing comorbidity and the age of the patient. In children with lymphoblastic leukemia, the five-year survival rate is up to 90%, among adults – about 40%.

With myeloid forms, the survival rate of children is up to 70%, the elderly – up to 12%.

The highest survival rates are for chronic lymphocytic leukemia, they reach 50-80%, and for myeloid leukemia – up to 90%. But if blast crises develop, survival prognosis is poor.

Sources:

G.S. Tumyan. Malignant lymphomas // Clinical oncohematology. Basic Research and Clinical Practice, 2009, pp.92-97.
O.Yu. Baranov. Acute leukemia // Clinical oncohematology. Basic Research and Clinical Practice, 2012.
A.A. Mayboroda. On the issue of diagnosing oncological diseases // Siberian Medical Journal, 2015, No. 2, pp. 128-134.
Taylor J, Xiao W, Abdel-Wahab O. Diagnosis and classification of hematologic malignancies on the basis of genetics // Blood. 2017 Jul 27;130(4):410-423. doi: 10.1182/blood-2017-02-734541. Epub 2017 Jun 9.
Godley LA, Shimamura . Genetic predisposition to hematologic malignancies: management and surveillance // Blood. 2017 Jul 27;130(4):424-432. doi:10.1182/blood-2017-02-735290. Epub 2017 Jun 9.

Information verified by an expert

Mikhailov Alexey Gennadievich
operating oncologist, doctor of the highest qualification category, Ph.D.
experience: 22 years

The information in the article is provided for reference purposes and does not replace the advice of a qualified specialist. Do not self-medicate! At the first sign of the disease, you should consult a doctor.

symptoms and signs, types, causes, diagnosis and treatment of blood cancer

Article content:

General data
Causes
Blood cancers
Manifestations of different forms of the disease
Diagnostics
Features of treatment
Forecasts

General information

These pathologies are divided into three large groups:

Leukemia – systemic lesions of hematopoiesis and peripheral blood cells. They do not have a specific localization of the tumor focus.
Lymphomas – lesions of the lymphatic system, in which there is one or more pathological foci where cancerous lymphocyte cells are found.
Paraproteinemic hemoblastoses .

Leukemias involve the bone marrow in the process, which in cancer produces immature hematopoietic cells – blasts. Lymphomas affect the lymph nodes and some of the internal organs.

For a long time, pathology can be hidden, and the symptoms of hemoblastoses are similar to each other, so cytogenetic analyzes play a decisive role in diagnosis.

Causes of blood tumors

various types of ionizing radiation;
presence of hereditary pathologies;
exposure to chemical carcinogens, including certain types of drugs;
exposure to viruses (Epstein-Barr or T-lymphotropic virus generations) Source:
Godley LA, Shimamura A
Genetic predisposition to hematologic malignancies: management and surveillance //
Blood. 2017 Jul 27;130(4):424-432. doi: 10.1182/blood-2017-02-735290. Epub 2017 Jun 9 .

Types of blood cancer

In addition to the division into three large types – leukemia, paraproteinemic hemoblastoses and lymphomas, within each group there is a division into types of tumor processes.

Lymphomas are divided into:

Hodgkin’s (they are also lymphogranulomatosis), which have several subtypes depending on the absence or presence of special cells in the affected tissues – Sternberg-Reed.
Non-Hodgkins . According to the degree of malignancy, they can be low-, medium- or high-malignant. Source:
G.S. Tumyan
Malignant lymphomas //
Clinical oncohematology. Fundamental research and clinical practice, 2009, p.92-97.

Leukemias are divided into two types:

Acute , in which the lesion affects blasts or immature hematopoietic cells.
Chronic , involves mature cells at different stages of formation. For chronic, the appearance of blast crises is typical, when the clinic resembles an acute leukemic process.

In the group of acute leukemias, according to the type of affected cells, there is a division into:

Myeloblastic forms of which should normally form platelets, erythrocytes or leukocytes.
Lymphoblastic forms . Lymphocyte precursors of T- and B-forms are affected. Source:
O.Yu. Baranova
Acute leukemia //
Clinical oncohematology. Basic Research and Clinical Practice, 2012.

In chronic leukemia there is a division into forms:

Lymphocytic leukemia , in which B-lymphocytes are affected and their mutant forms are formed.
Myelocytic leukemia in which the granulocytic series of leukocytes suffers.

In paraproteinemic hemoblastoses, abnormal, monoclonal immunoglobulins, abnormal proteins produced by mutated cells, accumulate in the blood. This type of blood cancer includes:

Myeloma with damage to the generation of lymphocytes, anemia, damage to bone tissue and excretion of paraprotein in the urine, damage to the kidneys.
Waldenström’s macroglobulinemia is a cancer that leads to a sharp change in blood viscosity due to an abnormal amount of protein and damage to organs, including vital ones.
Heavy chain disease with accumulation in the blood of large protein molecules with different chains (usually alpha or gamma), less often delta or mu forms. Possible damage to different parts of the digestive tube, severe baldness with cachexia, fever, lesions of the lymph nodes.

Manifestations of different types of cancer

Signs of hemoblastosis largely depend on the stage of the disease. In cancer, several stages are distinguished with key characteristics:

Initial – malignant cells have just formed, they divide and accumulate, there are no external symptoms and manifestations.
Expanded – typical symptoms of a particular type of blood cancer occur, changes in the blood are the most characteristic.
Terminal – drugs stop helping, the process of normal hematopoiesis is completely suppressed, only cancer cells are formed.
Remission stage (complete or incomplete possible) with restoration of normal parameters in the peripheral blood and in the bone marrow.
Relapse stage – the return of symptoms of the disease with the appearance of cancer cells after a certain period of remission.

frequent colds with complications due to a decrease in the immune functions of the blood;
anemia that does not respond to therapy;
soreness of bones and large joints;
constant weakness, fatigue, pallor.

Leukemia will typically present with anemia and weakness, attacks of dizziness, bruising and hemorrhage in the whites of the eyes, bleeding gums, severe pain in the bones, enlarged liver with spleen.

Lymphoma is also characterized by lymphadenopathy (permanent and pronounced enlargement of the lymph nodes), night sweats, fever due to weight loss, skin itching.

Myeloid disease is characterized by bone fractures, increased thirst with large amounts of urine, kidney damage, anemia and frequent colds, bleeding from wounds and injection sites.

Diagnosis of blood cancer

Diagnosis is based on laboratory tests to detect cancer cells in peripheral blood and bone marrow aspirates.

For paraproteinemic hemoblastoses, blood and urine tests are important, including detection of specific proteins, MRI or CT, and ultrasound of the abdomen and kidneys.

Features of the treatment of blood cancer

In the treatment of blood cancer, various methods are used that have a fairly high efficiency. Shown:

Surgical treatment for certain types of cancer.
Chemotherapy.
Bone marrow stem cell transplant.
Radiation methods of treatment.
Palliative care (blood, erythrocyte, platelet transfusions).

Treatment of hemoblastoses is accompanied by side effects, so it is necessary to carry out supportive, symptomatic therapy and a long course of rehabilitation.

Predictions

With myeloid forms, the survival rate of children is up to 70%, the elderly – up to 12%.

The highest survival rates are for chronic lymphocytic leukemia, they reach 50-80%, and for myeloid leukemia – up to 90%. But if blast crises develop, survival prognosis is poor.

Sources:

G.S. Tumyan. Malignant lymphomas // Clinical oncohematology. Basic Research and Clinical Practice, 2009, pp.92-97.
O.Yu. Baranov. Acute leukemia // Clinical oncohematology. Basic Research and Clinical Practice, 2012.
A.A. Mayboroda. On the issue of diagnosing oncological diseases // Siberian Medical Journal, 2015, No. 2, pp. 128-134.
Taylor J, Xiao W, Abdel-Wahab O.

Lymphoma rib cage pain. Primary Rib Non-Hodgkin’s Lymphoma: A Rare Case of Bone Lymphoma Mimicking Male Breast Cancer

Understanding Primary Lymphoma of Bone: A Rare Entity

Clinical Presentation: When Lymphoma Masquerades as Breast Cancer

Diagnostic Journey: Unraveling the Mystery

Treatment Approach: Balancing Local Control and Systemic Therapy

Disease Progression and Prognosis: A Challenging Outcome

Lessons Learned: Improving Diagnosis and Management

Future Directions: Advancing Treatment for Primary Bone Lymphomas

Differential Diagnosis: When Bone Lesions Aren’t What They Seem

The Role of Imaging in Diagnosis and Staging

Histopathological Features: Clues to Diagnosis

Treatment Challenges: Balancing Local and Systemic Approaches

Prognostic Factors in Primary Bone Lymphoma

The Importance of Multidisciplinary Care

Emerging Therapies: Hope for Improved Outcomes

Primary lymphoma of bone originating in a rib

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Unusual primary osseous Hodgkin lymphoma in rib with associated soft tissue mass: a case report and review of literature | Diagnostic Pathology

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Authors’ original submitted files for images

Authors’ original file for figure 1

Authors’ original file for figure 2

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About this article

symptoms and signs, types, causes, diagnosis and treatment of blood cancer

Article content:

General information

Causes of blood tumors

Types of blood cancer

Manifestations of different types of cancer

Diagnosis of blood cancer

Features of the treatment of blood cancer

Predictions

symptoms and signs, types, causes, diagnosis and treatment of blood cancer

Article content:

General information

Causes of blood tumors

Types of blood cancer

Manifestations of different types of cancer

Diagnosis of blood cancer

Features of the treatment of blood cancer

Predictions

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