Paraneoplastic multicentric reticulohistiocytosis: a clinicopathologic challenge

Multicentric Reticulohistiocytosis (MRH) is a rare, systemic non-Langerhans cell histiocytosis (non-LCH) with prominent joint and skin manifestations. It is mostly self limiting. However, 15-30% of the cases are associated with malignancy and carry a poor prognosis. We report the case of a 42-year-old man who presented with multiple reddish-brown papules that on biopsy showed aggregates of oncocytic histiocytes with several multinucleate giant cells. Immunostains were positive for CD 68, CD 45 and were negative for S-100, CD1a. An impression of multicentric reticulohistiocytosis (MRH) was made, with the recommendation to screen for malignancy. Electron microscopy of the skin lesions showed features consistent with non-Langerhans cell histiocytosis. The patient was later diagnosed with acute myeloid leukemia at a follow-up visit several months later. Thus, it appears prudent to screen and follow-up adults with MRH, to identify an underlying malignant condition.     

Epithelioid trophoblastic tumor of uterus presenting as an ovarian mass: a diagnostic and therapeutic dilemma

Epithelioid trophoblastic tumor (ETT) is a rare gestational trophoblastic tumor and often poses a diagnostic and therapeutic challenge to the involved clinicians. We report a case of epithelioid trophoblastic tumor in a young woman which involved the uterus, parametrium and the right ovary. Misdiagnosis as a choriocarcinoma led to improper treatment and progressive disease. Microscopically it revealed a relatively monotonous population of epithelioid cells arranged in nests with hyaline-like matrix surrounding the tumor cells. Differential diagnosis between placental site trophoblastic tumor and carcinoma was ruled out based on histology and immunohistochemistry. The patient developed lung and brain metastasis after 10 months and is alive with disease 1(1/2) years thereafter and is taking palliative chemotherapy. The patient had beta-HCG level of 85.1 mIU/mL at the time of diagnosis; but just before metastasis, the levels rose. Awareness of the histological features of ETT is essential to avoid misdiagnosis, as it represents a tumor which is primarily treated by surgery rather than with chemotherapy.     

Ultrastructure in resolving a diagnosis of poorly differentiated clear cell sarcoma of soft parts in an adolescent male

Clear cell sarcoma of soft parts is a rare tumor in children and it requires a high index of suspicion for accurate diagnosis. Early diagnosis leads to radical surgical excision and limits the aggressive behavior of this tumor. We report a case of a 12-year-old boy with a recurrent soft-tissue tumor in the scalp, misdiagnosed on three occasions as epitheloid sarcoma owing to the poorly differentiated appearance of cells. In spite of focal S-100 expression, this tumor was not recognized as a tumor of melanocytic origin till melanosomes were demonstrated on electron microscopy (EM). Detection of melanosomes on electron microscopy helped in clinching the histology diagnosis, reiterating the definite role of EM in diagnosing these tumors. Failure to accurately diagnose this tumor resulted in institution of preoperative chemotherapy, delayed surgical excision, tumor progression and death of patient within a year and half of presentation.     

Metastasis of humeral osteosarcoma to the contralateral breast detected by <Superscript>99m</Superscript>Tc-MDP skeletal scintigraphy

A rare case of osteogenic sarcoma (OGS) of the left humerus metastasizing to the right breast is presented. This lesion was detected by serendipitous observation on ^99mTc-MDP skeletal scintigraphy that demonstrated a rounded focus just outside the skeletal outline of the right chest. The focus remained at a similar location on repeat postwash view. Clinical examination revealed a breast mass corresponding to the site of uptake, and a biopsy of this mass revealed it to be a metastasis of osteogenic sarcoma. The present case demonstrates a rare site of metastasis from OGS of the left humerus to the contralateral breast and hence is reported here as an unusual case vignette.     

Primary angiosarcoma of the breast: observations in Asian Indian women

BACKGROUND: Primary angiosarcomas of breast are rare tumors, with a fatal outcome. MATERIAL AND METHODS: We studied histological prognostic factors and c-kit expression by immunohistochemistry (IHC) in 12 angiosarcomas accessioned at a cancer referral center in India. RESULTS: All patients had primary angiosarcoma; no case of secondary angiosarcoma was accessioned during the study period. Median age of patients was 24.5 years. Nine patients had intermediate grade tumors, one a well differentiated tumor and three patients had high-grade tumors. Interesting cases encountered included an epithelioid angiosarcoma and an angiosarcoma arising on the background of a biphasic tumor. Eight patients had lumpectomy, four mastectomy and two patients were given radiotherapy. Of the nine patients (seven type I/II and two high grade) with follow up, eight patients developed disseminated metastases within a year of presentation. The patient with well-differentiated angiosarcoma also died of metastasis albeit after a longer time. On IHC c-kit staining was weakly seen in two cases. CONCLUSION: Primary angiosarcoma was fatal in young Indian women even in lower grade tumors. The low expression of c-kit on IHC suggests that targeting this protein for therapy may not be successful in treating these tumors.