Extraventricular neurocytoma in a child mimicking oligodendroglioma: a diagnostic pitfall.

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.

[Epidemiology of extrapulmonary hydatid cysts: 265 Tunisian cases]. / Epidémiologie des kystes hydatiques extrapulmonaires : 265 cas en Tunisie.

AIM OF STUDY: The authors wanted to analyze the epidemiological features of extrapulmonary hydatid cysts and compare their results with those reported in literature. METHODS: Two hundred and sixty-five cases of extrapulmonary hydatid cysts collected from 1990 to 2007 were retrospectively studied. RESULTS: One hundred and one male and 164 female patients (sex ratio M/F=0.61 mean age 38.7 years) were included. In our series, hydatid cysts involved mainly the kidney (24.1%), the central nervous system (22.6%), the liver (19.6%) and the spleen (11.3%). CONCLUSION: Contrary to published data, our results show that hydatid cysts of the kidney and of the central nervous system are more frequent than hepatic location which ranks 3rd. Such unusual results may be due to a selection bias.

Cutaneous gamma-delta T-cell lymphoma arising in the setting of Behcet's disease.

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.

Extragastrointestinal stromal tumor of the urinary wall bladder: case report and review of the literature.

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.

[Primary amyloidosis of the spine presenting with acute paraplegia]. / Amylose primitive et étagée du rachis dorsolombaire compliquée d'une paraplégie.

Primary amylosis is rarely located in bone, exceptionally in the spine. The radiographic presentation is polymorphous. Magnetic resonance imaging is currently the best imaging technique. Spinal amyloidosis can exceptionally lead to cord compression requiring rapid surgical release.

Subependymomas: a clinicopathological study of 6 symptomatic cases.

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.

Canalicular adenoma of buccal mucosa.

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.

Intracranial medulloepithelioma. A case report and review of the literature.

Medulloepithelioma is a rare primitive neuroectodermal tumour of the central nervous system usually developing in childhood, displaying highly malignant behaviour, with early progression or recurrence. The Authors report a new case of intracranial medulloepithelioma removed from the right cerebellar hemisphere of a 13-year-old boy who showed unspecific signs of intracranial hypertension. Based on a review of the thirty seven cases reported in the literature, they analyse the clinical, histological, immunohistochemical, ultrastructural and therapeutic features of this rare nervous system tumour.

Huge cystic lymphangioma of the adrenal gland. A case report and review of the literature.

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.

[Subcutaneous phaeomycotic cyst of the ankle]. / Kyste phaeomycotique sous-cutané de la cheville.

Phaeohyphomycosis is a rare mycotic opportunist disease witch usually affects immunodepressed patients. Infection follows a traumatic inoculation of fungi into the skin with formation of a subcutaneous cyst. We report the case of a 20-year-old woman presenting with a subcutaneous, painless, and recurrent cystic lesion on the right ankle for 9 years. A surgical excision was performed. Histopathologic examination showed a granuloma with central abscess surrounded by a thick fibrous tissue. After staining with PAS, septate hyphae and spores were found in the granuloma, consistent with a phaeomycotic cyst.

[Hydatid cyst of the posterior fossa]. / Kyste hydatique de la fosse postérieure.

Hydatidosis is an endemic affection in Tunisia. Cerebral echinococcosis is a relatively rare entity accounting for only 1-2% of all hydatid cysts in humans. Extradural hydatid cyst of the posterior fossa is a very uncommon site for the disease. We report the case of a four-year-old child admitted for high intracranial pressure. Brain CT scan showed an extradural posterior fossa cyst without enhancement after contrast medium injection. Operative finding revealed a hydatid cyst. The histological examination of the tissue sample confirmed the diagnosis. The patient was given albendazole post operatively. She feels well six months later.

[Chordoid meningioma. Two cases]. / Le méningiome chordoïde. A propos de deux cas.

Chordoid meningioma is an uncommon histopathological variant of meningioma frequently confused with chordoma. Recurrence is not uncommon. The aim of our study is to present the clinical and pathological characteristics of this entity and a review of the literature. We report two cases of chordoid meningioma occurring in two women aged 47 and 63 years. Both women presented neurological signs without systemic disease. Radiological findings were temporoparietal and temporofrontal lesions with intense enhancement after contrast injection. Complete surgical resection was performed. Histopathologic findings were consistent with chordoid meningioma.

Primary cutaneous leiomyosarcoma: a histological and immunohistochemical study of 4 cases.

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.

[Carcinosarcoma of the urinary bladder. Report of three cases]. / Carcinosarcome de la vessie: a propos de trois cas.

BACKGROUND: Carcinosarcoma of the urinary bladder is a very rare neoplasm accounting for less than 0,05% of urothelial tumours is a biphasic malignant neoplasm with only 79 cases reported in the English literature. Histologically, it exhibits morphologic and/or immunohistochemical evidence of epithelial and mesenchymal differentiation with the presence or absence of heterologous elements. It usually involves aged adults after 60 years and it has a poor prognosis. AIM: We report three cases with an emphasis on unusual histologic features. CASES: Three male patients age 76 to 86 years were found to have polypoid masses in the urinary bladder. In all cases, microscopic examination revealed biphasic neoplasms with distinct mesenchymal and epithelial components. The two first cases were remarkable respectively by the presence of chondrosarcomatous and osteosarcomatous components.

[Testicular plasmocytoma: a case report]. / Le plasmocytome testiculaire: a propos d'un cas.

BACKGROUND: Extramedullary plasmocytoma is rare, with less than 50 reported cases and only 8 cases of primary testicular plasmocytoma. AIM: Report of a new case CASE: This report concerns a 65-year-old patient presenting an enlargement of the testis. The diagnosis of testicular plasmocytoma was made on histological examination. There was no evidence of bone marrow involvement or systemic signs of myeloma. This case will be studied with a review of the literature.

[Testicular lymphoma: report of 4 cases]. / Les lymphomes testiculaires: a propos de 4 cas.

BACKGROUND: Primary testicular lymphoma is the most common testicular malignancy in the elderly, account for 1% of all non Hodgkin lymphoma (NHL). The aim was to report from new cases of testicular lymphoma CASES: We report the only four cases of testicular lymphoma observed in a period of 15 years; all these cases were primary diffuse large cell B NHL. Disease course is usually aggressive, with widespread organ involvement. Retrospective analyses show a poor prognosis for this presentation.

[Rhinocerebral mucormycosis: A case report]. / Mucormycose rhinocérébrale: à propos d'un cas.

Mucormycosis is a rare and acute fungal infection which is frequently lethal, usually observed in non-controlled diabetic patients. The infection usually begins in the nose but it can invade the lung, the digestive tract, and the skin. Rhinocerebral mucormycosis accounts for 40 to 49% of mucormycosis cases. We report the case of a 53-year-old diabetic man, with chronic renal failure, presenting with rhinocerebral mucormycosis. Our patient was treated by an association of amphotericin B and surgical debridement. This observation provides on opportunity to recall clinical, histopathological, and therapeutic aspects of rhinocerebral mucormycosis.

[Renal actinomycosis: a case report]. / Actinomycose rénale: a propos d'une observation.

This report describes the case of a 26-year-old patient hospitalized for spontaneous abdominal pain. Palpation during clinical examination demonstrated a mass in the right abdomino-pelvic cavity. Radiological examination visualized the presence of grains suggestive of actinmycosis. The patient responded favorably to antimicrobial therapy. Actinomyces israeli is a gram-positive anaerobic bacterium that can cause tumor-like lesions usually on the face and neck and more rarely on retroperitoneal structures. Because renal involvement can require nephrectomy, diagnosis of renal actinmycosis must be made during pre-operative work-up. Ultrasound-guided needle biopsy of the lesion can achieve diagnosis and avoid nephrectomy since medical treatment using antimicrobial therapy is effective.