Management of a giant retroperitoneal leiomyoma: a case report.

BACKGROUND: Leiomyomas are benign tumors observed mainly in adult women. The retroperitoneum is a rare location for leiomyomas; almost 100 cases have been reported. Because retroperitoneal leiomyomas are paucisymptomatic and the tumor size at diagnosis is relatively large, surgical management is challenging. Regular follow-up is required because recurrence and malignant sarcomatous transformation have been described in a few cases. CASE PRESENTATION: We report a case of a 52-year-old North African woman with a 22-cm retroperitoneal leiomyoma. A preoperative embolization was performed 2 days before surgery. The clinical, therapeutic, and evolutive aspects of this rare entity are discussed. CONCLUSIONS: Despite its benignity, retroperitoneal leiomyoma is a challenging diagnostic, therapeutic, and evolutive condition. Surgeons must consider mainly the tumor's vascularization. Regular follow-up is mandatory because malignant transformation cannot be excluded.

Scrotal calcinosis: two case reports.

BACKGROUND: Scrotal calcinosis is a rare and benign condition. It usually gives rise to few symptoms, and the impact is mainly functional and aesthetic. It is considered part of dystrophic calcinosis cutis. Surgical management is the only curative approach, and recurrence has been described in few cases. CASE PRESENTATION: We report cases of two North African white patients with operated scrotal calcinosis. We describe the clinical and histological aspects as well as a pathogenic hypothesis and surgical management principles. CONCLUSIONS: A surgical approach to scrotal calcinosis must consider the aesthetic and functional aspects postoperatively. A complete excision prevents recurrence. Psychological support is required in association with surgery because the lesions are benign and concern an intimate part of the body.

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.

Hemangiopericytoma in the central nervous system. A study of eight cases.

Most hemangiopericytomas (HPC) are located in the musculoskeletal system and the skin, while the location in the central nervous system (CNS) is rare. The latter represents 2 to 4% in large series of meningeal tumors, thus accounting for less than 1% of all CNS tumors. In the central nervous system, tumors with a hemangiopericytomatous histolopathological pattern can be either hemangiopericytomas or solitary fibrous tumors. CNS-HPCs have a relentless tendency for local recurrence and metastases outside the CNS. Metastasis can also appear many years after adequate treatment of the primary tumor. We present a pathological study of eight patients with CNS-HPC and compare our results with corresponding published data. The CNS-HPC group consisted of three males and five females with a mean age of 36.75 years. The tumors were supratentorial in four cases, infratentorial in two cases, tentorial in one case and located in the spinal cord in the last one. Histologically, CNS-HPCs were similar to their soft tissue counterparts. One case demonstrated increased cellularity, marked nuclear hyperchromasia and marked cellular pleomorphism with infiltration of the cerebellum. All patients underwent surgery with gross-total resection in all cases. No patients received postoperative radiation therapy. Only four patients recurred locally after six, seven and eight months, and five years. Our study presents the pathological features of CNS-HPC as a distinct entity from both meningioma and solitary fibrous tumors. A comparative review of literature with our results is discussed.

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.

Primary central nervous system malignant melanoma: report of 5 cases.

Primary melanocytic neoplasms of the central nervous system consist of a spectrum of lesions ranging from well-differentiated melanocytoma to its overtly malignant counterpart, melanoma. Clinicopathologic features of 5 cases of primary central nervous system malignant melanoma were studied to define histologic appearances, diagnostic criteria, and the clinical behavior of lesions along this spectrum. There were three women and two men (mean age was 35 years) with symptoms of increased intracranial pressure. Radiological findings showed a solitary intra parenchymal lesion with variable enhancement after gadolinium administration. All patients underwent surgical treatment. Histologic examination revealed primary melanoma.

Desmoplastic small round cell tumor of the abdomen. Report of two cases.

Desmoplastic small round cell tumor (DSRCT) is a rare clinicopathological entity individualized in 1989. Its etiopathogenesis is still unknown, and diagnosis can be achieved only by immunohistochemistry and cytogenetic studies. The objective of this work is to report two new cases of DSRCT and to review the literature to clarify its epidemiological, clinical and pathological aspects.

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.

[Benign sacrococcygeal teratoma in an adult]. / Tératome sacrococcygien bénin de l'adulte.

Sacrococcygeal teratomas are rare congenital tumors generally discovered at birth. Exceptionally these tumors are observed in adults. Radical resection must be performed to avoid potentially malignant recurrence even if the primary lesion was benign. We report a case of a benign sacroccygeal teratoma diagnosed in a 25-year-old male and discuss clinical features, pathological considerations and treatment.

[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.