Composite Hemangioendothelioma: Report of a Rare Case.

Composite Hemangioendothelioma (CHE) is a new entity classified under the Heman gioendothelioma (HE) group of tumours. It is an extremely rare vascular neoplasm of low to moderate malignancy. Very few cases have been described in the literature. We herein report a case of CHE arising in a 31-year-old male who presented with a history of painless slow growing swelling on upper back since 1year. Clinically, the surface of firm and mobile swelling of 1.5x1.5cm showed focal ulcerations. The histopathological findings were consistent with the diagnosis of composite hemangioendothelioma. The patient underwent wide local excision and at the end of five months of follow up there were no features of local recurrence or metastasis. To the best of our knowledge, this is the first reported case of CHE from India.

Effectiveness of Pooled Platelet Transfusion in Concordant and Discordant Groups among Dengue Patients.

INTRODUCTION: Dengue affects more than 50 million people per year and is one of the most common causes of severe thrombocytopaenia. Thrombocytopaenia is a common complication of dengue and other viral fevers apart from malaria, typhoid, leptospirosis, leukaemia and megaloblastic anaemia. A platelet count of <20,000/µl is characteristically seen in dengue haemorrhagic fever and dengue fever. It results from immune complex mediated platelet destruction or bone marrow suppression. Severe thrombocytopaenia <10,000/µl is one of the indications for prophylactic platelet transfusion therapy to prevent haemorrhage. AIM: To evaluate the effectiveness of transfusion of ABO compatible and ABO incompatible pooled platelet units in severe thrombocytopaenia cases. MATERIALS AND METHODS: In this study ABO compatible and incompatible pooled platelet units were transfused to serologically confirmed dengue cases having thrombocytopaenia with or without bleeding manifestations. Each of the adult patients received 4-6 units of pooled platelet concentrates prepared from random donor whole blood suspended in plasma for severe thrombocytopaenia. Pre and post transfusion platelet counts were compared. Children aged less than 12 years, pregnant women and patients with splenomegaly those on ayurvedic and homeopathic therapy, recipients of packed red cells on the same day of platelet transfusion and recipients of multiple platelet transfusions within 24 hours were excluded from the study. RESULTS: The median post transfusion platelet increments (PPI) and corrected count increments (CCI) at 4hour post transfusion were 25,000/µL (5,000-80,000/µL) and 18,000/µL (range 8,000/µL- 47,500/µL) respectively among the responders. Median PPI and CCI at 24 hours were 45,000/µL and 28,863/µL among the responders. The median CCI at 4 hour post transfusion among the non-responders was 850/µL and at 24hours was 1,425/µL. At 24 hours responders showed significantly higher PPI as compared to non responders. The average platelets transfused were 4units in case of responders and 8 units in case of non-responders. CONCLUSION: ABO identical and compatible pooled platelet transfusions were more successful in increasing the post transfusion platelet counts as compared to ABO incompatible pooled platelets.

Pseudoangiomatous variant of spindle cell lipoma: Report of a rare case.

Pseudoangiomatous spindle cell lipoma is a rare variant of spindle cell lipoma. It exhibits irregular and branching spaces with villiform connective tissue projections, leading to formation of a characteristic angiomatoid pattern in addition to benign spindle cells and mature adipocytes. Recent studies have shown that these branching spaces are lined by endothelial cells and express markers. Therefore, the term "angiomatous variant" instead of "pseudoangiomatous" has been proposed for these lesions. We describe one of such lesions in the thumb of a young female. To the best of our knowledge, this is the first reported case of pseudoangiomatous variant of spindle cell lipoma from India.

Eccrine Porocarcinoma of the Scalp: A Rare Case Report with Review of Literature.

Eccrine porocarcinoma (EPC) is a potentially lethal neoplasm of the skin that arises from the intraepidermal portion of the eccrine sweat glands. It was previously known as eccrine adenocarcinoma or malignant eccrine poroma. It commonly occurs between 60 to 80 years of age, usually arising from hands and feet. However, few case reports on involvement of rare sites such as scalp, face and eyelids are present in literature. We herein report an unusual case of porocarcinoma arising on the right parieto occipital region of scalp in a 29-year-old young female patient. To the best of our knowledge, there have been fewer than 20 cases of porocarcinoma arising on scalp with fewer than 10 cases seen in younger age group reported previously in the literature.

Solitary Angiokeratoma: Report of Two Uncommon Cases.

Angiokeratomas are rare benign vascular skin lesions arising in isolation or in groups of multiple lesions, as solitary cutaneous forms or generalized systemic forms. They are ectasias of dermal capillaries with an acanthotic and hyperkeratotic epidermis. They can occur in both healthy individuals and in those with underlying systemic disease due to inherited enzyme deficiency or other acquired predisposing factors. The identification and reporting of these lesions is important as patients with these lesions should be evaluated to rule out underlying pathogenic conditions. We report two rare cases of isolated solitary cutaneous angiokeratoma occurring in two patients.

Basal cell adenoma of the parotid gland: Cytological diagnosis of an uncommon tumor.

Basal cell adenoma (BCA) is a rare benign epithelial tumor of the salivary gland, displaying monomorphic basaloid cells without a myxochondroid component, representing 1-3% of all salivary gland neoplasms seen predominantly in women over 50 years of age. It is uncommon in young adults. Cytodiagnosis of basaloid tumors chiefly basal cell adenoma of the salivary gland, is extremely challenging. The cytological differential diagnoses range from benign to malignant, neoplastic to non- neoplastic lesions. Histopathological examination is a must for definitive diagnosis, as these entities differ in prognosis and therapeutic aspects. We present a 22-years-old male with this uncommon diagnosis with a discussion on the role of cytological diagnosis. Fine needle aspiration cytology is a simple, minimally invasive method for the preoperative diagnosis of various types of neoplastic and non-neoplastic lesions. The knowledge of its pitfalls and limitations contributes to a more effective approach to treatment.

Nerve sheath myxoma: report of a rare case.

Nerve sheath myxoma defined by Harkin and Reed is an uncommon benign neoplasm with nerve sheath like features. It has several cytological and histological differential diagnoses. One such lesion is neurothekeoma, which can be differentiated using immunohistochemistry. In most of the previous reports nerve sheath myxoma and neurothekeoma were considered synonymous and were often confused for one another. This case report separates the two using immunohistochemistry. Also, the cytological features of nerve sheath myxoma are not well documented in the past. This case report attempts to display the cyto-morphology of nerve sheath myxoma. We report a rare case of nerve sheath myxoma diagnosed on cytological features confirmed by histopathology and immunohistochemistry in a 32-year-old lady who presented with an asymptomatic nodule over the left cervical area and discuss its cyto-histological mimics.

Isolated intracranial rosai-dorfman disease involving the meninges: report of a rare case.

Sinus histiocytosis with massive lymphadenopathy or Rosai-Dorfman disease (RDD) is a reactive condition of unknown etiology, characterised by a proliferation of histiocytes exhibiting emperipolesis of lymphocytes and plasma cells. It usually presents as bilateral painless cervical lymphadenopathy. Extranodal RDD without nodal disease is seen in 23% of the cases. Intracranial RDD occurs in less than 5% of the total number of patients with extranodal disease. Isolated intracranial RDD in the absence of nodal disease is exceptional. We report a case of Rosai-Dorfman disease with isolated intracranial involvement. A 38-year-old man came with long-standing headache and the magnetic resonance imaging (MRI) scan showed features of meningioma. The histopathology revealed sheets of histiocytes displaying emperipolesis. These histiocytes were S100 positive but CD1a and epithelial membrane antigen negative.

Cystic fibroadenoma: report of a rare case with review of literature.

Fibroadenomas with a predominant cystic change are called cystic fibroadenomas. These are extremely rare forms of fibroadenomas and only one case has been reported so far. They are classified under the category of complex fibroadenomas. Complex fibroadenomas are a rare variant of fibroadenomas occurring in elderly females. They are characterized by presence of one of the complex features along with the usual patterns of fibroadenoma such as cysts more than 3 mm, papillary apocrine metaplasia, or sclerosing adenosis. Patients with these lesions have higher chances of developing carcinoma of breast. We present a case of 35 years old lady with a freely mobile mass in the left breast diagnosed as cystic fibroadenoma after thorough histopathological examination of the lesion.

Soft tissue giant cell tumour of low malignant potential: a rare tumour at a rare site.

"Soft tissue giant cell tumour of low malignant potential" is considered as the soft tissue counterpart of osteoclastoma of the bone. It is a primary soft tissue tumour which is classified under the category of fibrohistiocytic tumours of intermediate malignancy.Seventy percent of the tumours involve the extremities and only about seven percent of them arise in head and neck region. They are composed of nodules of histiocytes in a vascular stroma, with multinucleated osteoclast-like giant cells positive for vimentin, smooth muscle actin (SMA), CD68 and Tarterate Resistant Acid Phosphatase (TRAP). We are presenting a case of a 75-year-old man who had a nodule on the ala of the nose. Histopathology showed a histiocytic lesion. Benign fibrous histiocytoma, plexiform fibrohistiocytic tumour, solitary reticulohistiocytoma and histioid leprosy were ruled out by using special stains and immunostains. Expression of smooth muscle actin and CD68 confirmed the diagnosis of a soft tissue giant cell tumour with a low malignant potential.