Shramana Mandal

Malignant peripheral nerve sheath tumor of the breast: case report

BackgroundMalignant peripheral nerve sheath tumor is a rare soft tissue sarcoma of ectomesenchymal origin. It is the malignant counterpart of benign soft tissue tumors like neurofibromas and schwannomas and may often follow them. Common sites include deeper soft tissues, usually in the proximity of a nerve trunk. Breast is an extremely rare location of this lesion and presentation as a breast lump in the absence of pain or previous benign neural tumor is even rarer.Case presentationA 38-year-old female presented with complaints of painless, hard breast lump for three months which was clinically suspected to be a ductal carcinoma with inconclusive fine needle aspiration cytology. Histopathology revealed a malignant spindle cell tumor which was confirmed to be malignant peripheral nerve sheath tumor on the basis of immunopositivity for vimentin, neurone specific enolase and S-100.ConclusionTo the best of our knowledge only six such case reports have been published in literature. The differential diagnosis of malignant peripheral nerve sheath tumor should be considered by the clinician as well as the pathologists in the work-up of a breast neoplasm as treatment and prognosis of this rare malignancy is different.

Cytomorphologic Spectrum in Paraganglioma

OBJECTIVE To study the detailed cytologic features of paragangliomas (PGs) and delineate features helpful in predicting behavior. STUDY DESIGN We performed retrospective analysis of 12 cases cytologically suggested as PG over a period of 10 years. Smears were reviewed for cellularity, pattern, shape, cytoplasm, nuclear features and background. Cytologic diagnosis was correlated with clinicoradiologic features, histologic features and immunocytochemistry. RESULTS Patient age ranged from 12 to 65 years, with male preponderance; they presented with a mass lesion. Sites involved were head and neck (5), retroperitoneum (5) and mediastinum (2). Smears were cellular; cells were round to oval, plasmacytoid and spindled and seen as scattered, in clusters, acinar and perivascular pattern. The most consistent feature was presence of pink granules. Pleomorphism was present in all but 1 case and less prominent in tumors with metastasis. Mitoses were more frequently seen in malignant cases. CONCLUSION Cytologic features of PG are diverse. Although there are certain main features, they are not diagnostic unless combined with data on site, histology and ancillary studies. Presence of granules, mild pleomorphism, frequent mitoses and necrosis needs careful evaluation for malignancy potential and warrants close follow-up.

Malignant fibrous histiocytoma following radiation therapy and chemotherapy for Hodgkin's lymphoma.

Malignant fibrous histiocytoma (MFH) originates from primitive mesenchymal cells and has the capacity for dual differentiation into histiocytes and fibroblasts. MFH occurring as a secondary malignancy following radio-chemotherapy is rare and its exact incidence is not yet known. Here we report a case of a 42-year-old man who developed MFH in his right knee over a period of more than 10 years after radio (44 Gy)-chemotherapy to treat Hodgkins lymphoma. After the diagnosis the patient did not return and was lost to follow-up. This is a rare case with unusual presentation, and it highlights the importance of the awareness and prevention of such secondary tumors.

Mucinous cystadenocarcinoma arising in mature cystic teratoma ovary and associated pseudomyxoma peritonei: report of a case

Pseudomyxoma peritonei (PMP) is most commonly associated with intra-abdominal spread of an appendiceal mucinous neoplasm and very rarely seen in cases of primary ovarian tumours. Mucinous adenocarcinoma arising in a mature cystic teratoma giving rise to PMP is even rarer. Extensive medlar search has revealed only nine cases; we are reporting tenth such case.

Adenoid cystic pattern in follicular variant of papillary thyroid carcinoma: a report of four cases

S. Mandal, and S. Jain
Adenoid cystic pattern in follicular variant of papillary thyroid carcinoma: a report of four cases

Evans syndrome: a study of six cases with review of literature

Abstract Evans syndrome is an uncommon condition characterised by simultaneous or sequential development of immune thrombocytopenia (ITP) and autoimmune haemolytic anaemia (AIHA) with a positive direct antiglobulin test (DAT) in the absence of a known underlying aetiology. The great majority of patients with Evans syndrome have a chronic relapsing course despite treatment, which is associated with significant morbidity and mortality. We reviewed the clinical and laboratory features of six patients with Evans syndrome. All patients had thrombocytopenia, bleeding symptoms and haemolytic anaemia with positive direct Coombs test at presentation. We discuss the aetiopathogenic, clinical, therapeutic and natural history of Evans syndrome.

Aggressive angiomyxoma of the vulva presenting as a pedunculated swelling.

Aggressive angiomyxoma is a rare, locally aggressive soft tissue tumor that has high propensity for local recurrence. It involves mainly the pelvis, vulva, perineum, vagina and urinary bladder in adult women in the reproductive age. Considering its locally aggressive nature, appropriate management and long-term follow-up is necessary. We describe a case of a 22-year-old young pregnant patient presenting with a large pedunculated swelling on the left labia majora.

Breast lump as an initial manifestation in acute lymphoblastic leukemia: an unusual presentation. A case report.

Abstract Breast lump as a presenting manifestation of acute lymphoblastic lymphoma (ALL) leukemia is extremely rare. We report a case of a young female who presented with a breast lump clinically suggestive of fibroadenoma. On fine needle aspiration cytology, it was diagnosed as lymphoid malignancy, later confirmed as ALL using hematological investigations.

Cutaneous Manifestations in Non-Hodgkin's Lymphoma

OBJECTIVE To examine and subtype cutaneous lymphoma specimens for diagnosis. STUDY DESIGN Aspiration smears from skin lesions and lymph nodes diagnosed as non-Hodgkins lymphoma (NHL) on cytology in 6 cases over a period of 1 year were reviewed. Two were follow-up cases of nodal lymphoma and were receiving chemotherapy, during which they developed skin lesions. In 4, the patients had cutaneous lesions as a presenting manifestation. Cytologic findings were correlated with histologic and hematologic findings and immunocytochemical markers for subtyping. RESULTS Patients ranged from 14 to 50 years, with equal sex ratio. All presented with 0.5-5 cm multiple nodular, ulcerated and fungating skin lesions at various body sites. The aspirate was satisfactory in all cases. Cytologically, all cases were diagnosed as NHL. They were then immunocytochemistry subtyped as various lymphomas. CONCLUSION Cutaneous lymphoma should always be considered in the presence of predominantly atypical lymphoid cells in smears from nodular and fungating skin lesions, even in the absence of a definitive clinical diagnosis.

Pulmonary Langerhans cell histiocytosis in children: A spectrum of radiologic findings

Pulmonary Langerhans cell histiocytosis (PLCH) is a well known entity in adults but is exceedingly rare in children. It is better described in adults than in children. We describe the current understanding of PLCH in children and a spectrum of radiological findings of PLCH in the paediatric population. On high resolution computed tomography (HRCT), PLCH may have variable appearance depending on the stage of disease, ranging from small interstitial nodular opacities to multiple thin/thick walled cysts (often bizarre in shape), eventually leading to marked parenchymal fibrosis and honeycomb pattern. CT finding of PLCH is similar in adult and paediatric populations with the exception that lung base near the costophrenic angle is spared in adults but almost always involved in children.