A rare case of Rosai-Dorfman disease presenting as solitary lesion of distal femur: A case report

Cases Series | Volume 8 | Issue 1 | JBST January – June 2022 | Page 07-10 |Ebin Rahman, Subin Sugath, Nita John.

DOI: 10.13107/jbst.2021.v08i01.67

Author: Ebin Rahman [1], Subin Sugath [1], Nita John [1]

[1] Department of Orthopaedic Oncology, Aster Medcity, Kochi, Kerala, India

Address of Correspondence
Dr. Ebin Rahman,
Senior Specialist, Department of Orthopaedic Oncology, Aster Medcity, Kochi, Kerala, India.
E-mail: rahmanebin@gmail.com

Abstract

Introduction: Rosai-dorfman disease (RDD) or sinus histiocytosis with massive lymphadenopathy is a rare histiocytic proliferative disorder of unknown etiology first identified and characterized by Rosai and Dorfman in 1969. The disease is characterized by painless cervical lymphadenopathy, fever, leukocytosis, elevated erythrocyte sedimentation rate, and hypergammaglobulinemia. <500 patients have been reported worldwide. Osseous involvement without lymphadenopathy occurs in <10% of patients and is usually multifocal. Extranodal solitary osseous lesions are just 4% of this subpopulation, no more than 50 such cases have been described in the literature. Here, we report a rare case of RDD presenting as solitary lesion of the distal femur without any evidence of lymphadenopathy and normal laboratory work up.
Case Report: We report the case of a 29-year-old female who presented with pain and swelling of distal femur of 2-month duration without any evidence of lymphadenopathy, with a normal laboratory workup. Radiographical evaluation showed an epiphyseal, osteolytic, and well-defined lesion with cortical break over the medial femoral condyle and soft-tissue extension into the medial patellar recess abutting the medial patellar retinaculum. Whole body positron emission tomography did not show any other sites of abnormal increased uptake. Intralesional extended curettage, bone grafting, bone cementing, and pinning were done. Histologically, the biopsied lesion demonstrated the classic histiocyte proliferation which exhibited emperipolesis (engulfment of lymphocyte or plasma cells by large histiocyte) which are CD-68, S-100 positive, and CD-1a negative on immunohistochemistry. At 2-year follow-up, the patient is symptom free, without any new osseous/extranodal/nodal lesions.
Conclusion: RDD rarely affects bone as a primary or even secondary form of the disease. It has relatively non-specific radiological appearance causing diagnostic challenges and may lead to erroneous diagnosis of a malignancy. Biopsy and histological examination by an experienced pathologist is mandatory for the final diagnosis. The ideal treatment ranges from wait and see policy to surgery, chemotherapy, and radiotherapy.
Keywords: Rosai-Dorfman disease, Extranodal, Osseous, Histiocytosis, Emperipolesis

Reference:
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How to Cite this article: Rahman E, Sugath S, John N | A rare case of Rosai-Dorfman disease presenting as solitary lesion of distal femur: A case report. | Journal of Bone and Soft Tissue Tumors | Jan-Jun 2022; 8(1): 7-10.

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Malignant Transformation Of Solitary Phalangeal Enchondroma To High Grade Chondrosarcoma- A Case Report

Cases Series | Volume 8 | Issue 1 | JBST January – June 2022 | Page 03-06 |Ebin Rahman, Subin Sugath, Nanda Kachare.

DOI: 10.13107/jbst.2021.v08i01.65

Author: Ebin Rahman [1], Subin Sugath [1], Nanda Kachare [2]

[1] Department of Orthopaedic Oncology, Aster Medcity, Kochi, Kerala, India

[2] Department of Pathology, Aster Medcity, Kochi, Kerala, India.

Address of Correspondence
Dr. Ebin Rahman,
Department of Orthopaedic Oncology, Aster Medcity, Kochi, Kerala, India.
E-mail: rahmanebin@gmail.com

Abstract

Introduction: Chondrosarcoma (CS) is a malignant neoplasm of mesenchymal origin characterized by the formation of cartilaginous matrix by neoplastic cells. It may arise as a primary lesion or occur secondarily at the site of a previous benign lesion such as enchondroma. Primary CS is rare in the hand with a frequency of <0.5–3.2% of all CS. Malignant transformation of a benign solitary enchondroma to secondary CS, especially in the hand, is extremely rare. This transformation is seen more often in multiple enchondromatosis than in solitary lesions, but even this event is rare in the hands. Here, we report a case of solitary enchondroma of proximal phalanx of middle finger initially treated with intralesional curettage and bone grafting, later recurring to high grade (Grade 3) CS.
Case Report: We report the case of 44-year-old lady presenting with pain and progressive fusiform swelling of the entire right middle finger of 4 months duration. She had a similar history 5 years back for which she had underwent intralesional curettage and bone graft substitute application. Curetted specimen was reported as enchondroma. Radiographical evaluation showed an expansile, intramedullary, and lytic lesion severe cortical destruction over proximal phalanx of the right middle finger. Magnetic resonance imaging detailed the soft-tissue component. She underwent a core needle biopsy which reported as low-grade CS arising from enchondroma. Resection of middle ray including distal 2/3 of 3rd metacarpal was performed. Excised specimen was reported as high-grade CS (Grade 3) with tumor free margins. At 2-year follow-up, she is symptom free without any local recurrence or distant lesions.
Conclusion: Diagnosis of CS in the hand can pose problems to clinicians and pathologists alike. To definitely set a diagnosis of CS in the hand, comparison of histological and radiological finding is paramount. CS are malignancies resistant to both radiation and chemotherapy. CS of the hand is characterized by local recurrence and very low metastatic potential. Hence, wide excision is the recommended approach.
Keywords: Phalanx, Enchondroma, Chondrosarcoma, High grade, Amputation.

Reference:
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How to Cite this article: Rahman E, Sugath S, Kachare N. | Malignant transformation of solitary phalangeal enchondroma to high grade chondrosarcoma – A case report. | Journal of Bone and Soft Tissue Tumors | Jan-Jun 2022; 8(1): 3-6.

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