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Extraventricular neurocytomas are rare neuronal tumours that have been included in the 2007 WHO classification as a variant of central neurocytoma. They arise outside the ventricles, usually within the cerebral hemisphere,s but also in other regions throughout the neuraxis. The morphological overlap of these tumours with oligodendroglioma often poses diagnostic difficulty. Herein, a case of extraventricular neurocytoma in a 4-year-old girl is reported that mimicked histologically oligodendroglioma. The authors describe the clinicopathological features of this rare entity with special emphasis on differential diagnosis.

Background: Subependymomas are rare, slow-growing, ependymal neoplasms that commonly occur in the fourth or lateral ventricles., Objective and Design: A retrospective study of 6 histologically proven subependymomas was undertaken to analyse their clinicopathological characteristics., Results: There were five male and one female patients ranging in age from 11 to 50 years (mean 35.8 years). All patients were symptomatic at diagnosis. The most common clinical presentations included headache (n=6) and vomiting (n=3). Tumours were located in the lateral ventricle in five cases and in the fourth ventricle in one case. Magnetic resonance imaging detected obstructive hydrocephalus in all cases. Five patients underwent gross total resection and one patient had subtotal excision of the tumour. Histologically, all tumours were characterised by clustering of isomorphic cells arranged against a fibrillary background. Focal cystic degeneration was seen in 5 tumours. During the follow-up period, which ranged between 2 months and 10 years, all patients were symptom-free with no evidence of recurrence.

Most mesenchymal tumours of the gastrointestinal tract are now referred to as gastrointestinal stromal tumours (GISTs). These tumours typically express c-kit (CD117) and CD34; 30-50% are (often focally) positive for alpha-smooth muscle actin, and all are negative for desmin and S100 protein. Recently, mutations in exon 11 of the c-kit gene have been identified as a molecular genetic marker for the subset of GISTs. In this report, we describe a mesenchymal tumour removed from the pelvic cavity of a 34-year-old man. The tumour was strongly attached to the external wall of the urinary bladder. The neoplasm grossly resembled a leiomyoma, and was histologically composed of sheets of spindle cells with a dense collagenous background. The mitotic activity was low (less then 1 per 50 high-power fields). Immunohistochemically, tumour cells were negative for alpha-smooth muscle actin and desmin and positive for CD117 and CD34. This case illustrates that tumours which are phenotypically and genotypically similar to GISTs may present in sites other than the tubular gastrointestinal tract.

Cutaneous gamma-delta T-cell lymphoma (CGD-TCL) is an uncommon lymphoma composed of a clonal proliferation of mature activated gamma-delta T-cells expressing a cytotoxic phenotype. Malignant lymphoma is rarely associated with Behcet's disease, as only 12 cases have been reported in the literature, including a case of cutaneous T-cell lymphoma. In this report, the authors present a new case of CGD-TCL emerging in the course of Behcet's disease in a 40-year-old man. Diagnosis of CGD-TCL was established based on the combination of clinical, histological, immunophenotypical and molecular findings. Through a review of the current literature, the authors analyse the unique clinicopathological, molecular and immunohistochemical features of this rare cutaneous lymphoma.

Background: Renal epithelioid angiomyolipoma is a recently recognized variant of angiomyolipoma, closely simulating renal cell carcinoma both clinically and histologically. Only a relatively small number of cases of epithelioid angiomyolipoma of the kidney have been reported., Aim: To highlight clinicopathological features of this rare tumour., Observation: We report herein a new case of renal epithelioid angiomyolipoma in a 38-year-old male with no stigmata of tuberous sclerosis. The tumour was composed of diffuse sheets of epithelioid cells, small numbers of adipocytes and occasional blood vessels. Immunohistochemically, neoplastic cells were immunoreactive for HMB-45, but negative for cytokeratin. The patient showed no evidence of recurrence or metastatic disease one year after radical nephrectomy., Conclusions: Epithelioid angiomyolipoma may be locally aggressive and can metastasise; therefore, long-term post-operative follow-up is mandatory.

Primary cutaneous leiomyosarcoma is an uncommon malignant neoplasm with a predilection for the lower extremities. A retrospective study of 4 cases was undertaken to analyse the clinicopathological characteristics and immunohistochemical profile of these neoplasms with emphasis on prognosis. Two male and 2 female patients aged between 49 and 80 years presented with painless tumours involving the lower lip, the chin, the scrotum and the shoulder. Histological examination of the initial biopsy specimen established a diagnosis of cutaneous leiomyosarcoma. All cases co-expressed smooth muscle actin and vimentin regardless of primary tumour site. Wide surgical excision of the tumour was performed in only 3 cases, and the remaining patient refused further treatment. Of the patients undergoing surgical intervention, local recurrence occurred in one case. No metastases were observed. Long-term follow-up of patients with cutaneous leiomyosarcoma is mandatory to detect local recurrence and distant metastases that can occur even years after the initial excision.

Canalicular adenoma is a benign tumor which comprises 1% of salivary gland neoplasms and 4% of minor salivary gland tumors. It occurs in the upper lip mucosa in about 90% of cases. The next most common location is the buccal mucosa (9.5% of tumors). We present herein a new case of canalicular adenoma of buccal mucosa involving a 74-year-old man. He was suffering of a slowly growing and painless nodule of the right buccal mucosa. The treatment was surgery and histological findings were consistent with the diagnosis of canalicular adenoma. No recurrence was noted one year later.

Cystic lymphangioma of the adrenal gland is a rare and benign lesion, most often found incidentally during abdominal imaging studies, abdominal surgery or at autopsy. Preoperative diagnosis is difficult. Diagnosis is histological. We report a 22-year-old female presented with abdominal pain. Laboratory findings were normal. Radiological examination revealed a 35 cm left adrenal cyst. The cyst was resected and the diagnosis of cystic lymphangioma of the adrenal gland was histologically made. In presenting this case and reviewing the literature, we seek to make better known the epidemiologic, the clinicopathologic presentation and the prognosis of this rare entity.

Most of the sinonasal tumours are of epithelial origin. Smooth muscle tumours are extremely rare, they account for less than 2.5% of the mesenchymal neoplasms of the sinonasal tract and the nasopharynx. Fewer than 30 cases of primary leiomyogenic tumours have been reported in the sinonasal tract with almost an equal frequency of benign and malignant types. We report a case of a rare leiomyoma of the nasal cavity. The pathological and the clinical characteristics of this tumour are discussed.

Granulocytic sarcoma is a form of extramedullary leukaemia. The intraparenchymal localisation is extremely rare. We report a case of cerebellar granulocytic sarcoma occuring in a 43 years old woman without any precedent medical history. The diagnosis of granulocytic sarcoma was established by neoplastic cells findings through morphological and immunohistochemical studies. The patient died few days after surgery. There are still no conclusive treatment strategies for this entity; however, early antileukemic chemotherapy seems to lower the probability of developing systemic disease and thus prolong survival.

The granular cell tumor is an uncommon tumor that usually appears as a solitary small nodular growth and runs a benign course. It occurs widely throughout the body, but is rarely described in the abdominal wall. The authors report a case of malignant granular cell tumor which was arising in anterior abdominal wall of a 67-year-old woman. Malignant variant is rare and the abdominal wall site is extremely uncommon. Regarding this clinical case and the literature the authors purpose to review the criteria of malignancy.