Kajal Kiran Dhingra

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.

Unsuspected multiples myeloma presenting as bilateral pleural effusion – a cytological diagnosis

Background Multiple Myeloma presenting as a pleural effusion is extremely rare. It is usually a late complication and is associated with a poor prognosis. Case Presentation A 40-year-old male presented with dyspnea and fever of six months duration. Clinical diagnosis of pulmonary tuberculosis was considered. X-ray chest showed bilateral pleural effusion. Pleural cytology revealed numerous plasma cells, some of which were binucleated and atypical. Cytological differential diagnosis included: Myelomatous effusion and Non-Hodgkins Lymphoma deposit (Immunoblastic type). Bone marrow biopsy, serum protein electrophoresis and bone scan confirmed the diagnosis of multiple myeloma (Plasmablastic type). Conclusion Myelomatous pleural effusion as an initial presentation although extremely rare, should always be considered in presence of atypical plasma cells irrespective of age.

Demodex-associated dermatologic conditions--A coincidence or an etiological correlate. Review with a report of a rare case of sebaceous adenoma.

Demodex folliculorum is a saprophytic mite of the human pilosebaceous unit with a prelidiction for facial skin and eyelashes. Infestation occurs frequently without symptoms; however, suppurative or granulomatous inflammation may be seen with high mite density. Skin lesions, such as rosacea, pityriasis, and blepharitis, have been attributed to Demodex; however, its role in sebaceous adenoma (SA) has not been reported in extensively searched medical literature. We report this rare case and review the skin lesions associated with Demodex folliculorum.

Morphological findings in bone marrow biopsy and aspirate smears of visceral Kala Azar: a review.

CONTEXT Visceral leishmaniasis (VL) is endemic in India and may simulate and cause many hematological disorders like pancytopenia, myelofibrosis, myelodysplasia and hemophagocytosis. AIMS The study aims to investigate the hematological manifestation of Visceral Leishmaniasis and associated changes that may be observed in bone marrow aspirate smears and biopsy which may warn a pathologist of possible infections. SETTINGS AND DESIGN This is a retrospective study of 18 VL cases on B (b) one marrow aspirate and biopsy in the department of Pathology in a tertiary care teaching hospital in New Delhi. METHODS AND MATERIAL Giemsa stained slides of bone marrow aspirates and hematoxylin and Eosin stained biopsy slides were reviewed in detail by two competent pathologists. All the findings were tabulated and discussed and comparisons made with the previous similar studies. RESULTS Hyper cellular marrow, increased lymphocytes and plasma cells, marrow granulomas, hemophagocytosis, myelofibrosis, myelodysplasia and gelatinous transformation of the marrow were notable features the presence of which together or individually should caution a pathologist to search for Leishman Donovan (LD) bodies in patients especially in a non-endemic zone in a tropical country.

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.

Bone marrow angiogenesis in aplastic anemia – a study of CD 34 and VEGF expression in bone marrow biopsies

Abstract Introduction: Bone marrow function and the growth of hemopoietic cells depends on an intact microvasculature. A pivotal regulator of angiogenesis is vascular endothelial growth factor (VEGF). Our study assesses VEGF expression and microvessel density (MVD) in the bone marrow of patients with aplastic anemia (AA). Materials and method: Bone marrow specimens from 25 patients with AA and 15 controls were studied. MVD was calculated on sections stained immunohistochemically for CD34. Subsequently, all the cases were studied for VEGF expression. Results: Bone marrow MVD in patients with AA was significantly lower than that in controls (p<0·01). There was a significant MVD difference between severe AA and moderate AA (p<0·05). VEGF expression was also significantly lower in AA cases compared to controls (p<0·05). Conclusion: Our data show that AA is associated with reduced angiogenesis and reduced VEGF expression. Defective angiogenesis may result in or aggravate bone marrow aplasia in AA patients. There are limited studies on this aspect. More studies to confirm the present hypothesis might pave the way for new treatment options in AA.

A rare coexistence of concurrent breast hemangioma with fibroadenoma: a case report

We report the case of a 38-year-old Asian, Indian female with capillary hemangioma breast in coexistence with the commonly occurring fibroadenoma. Clinical examination of the breast revealed a 4 cm diameter lump. Mammography revealed a well defined slightly hypoechoic lesion with smooth contours. A lumpectomy was performed. Histopathology confirmed the diagnosis of a completely encapsulated fibroadenoma coexistent with a capillary hemangioma in the adjacent breast tissue. The rarity of literature on breast hemangioma especially capillary type with coexisting fibroadenoma deserves mention.

Rare growth of a psammomatous meningioma in a mature ovarian teratoma: a case report.

The presence of meningothelial elements along with abundant psammoma bodies indicates the presence of a benign meningioma within a teratoma. Recognition of meningioma arising in the setting of a teratoma is of prognostic significance, depending on the nature of this component and its spread beyond the organ of origin. We report a case of psammomatous meningioma in which meningothelial cells and psammoma bodies were both abundant and diffuse, allowing for the diagnosis of a meningioma.

Langerhans cell histiocytosis masquerading as tuberculosis: a diagnostic dilemma resulting in inappropriate anti-tubercular therapy.

Langerhans cell histiocytosis is known to mimic many other conditions. We present two patients where anti‐tubercular therapy was instituted when clinical and radiological features suggested tuberculosis. The correct diagnosis of histiocytosis was reached only on further work‐up including immunohistochemistry following un‐responsiveness to treatment. In retrospect it was felt that an inordinately high index of suspicion for tuberculosis, clinical and radiological overlap between the two entities, an urgency to start empirical therapy in a rapidly deteriorating patient and pathological similarities between epithelioid histiocytes and pathologic Langerhans cells led to the diagnostic and therapeutic errors. Pediatr Blood Cancer 2009;53:111–113.