Retroperitoneal perivascular epithelioid cell tumours: A case report and review of literature.

BACKGROUND: The perivascular epithelioid cell tumour (PEComa) family of tumours mainly includes renal and hepatic angiomyolipomas, pulmonary lymphangioleiomyomatosis and clear cell "sugar" tumour of the lung. Several uncommon tumours with similar morphological and immunophenotypical characteristics arising at a variety of sites (abdominal cavity, digestive tract, retroperitoneum, skin, soft tissue and bones) are also included in the PEComa family and are referred to as PEComas not otherwise specified. CASE SUMMARY: We present a 37-year-old female patient who underwent resection of an 8.5 cm × 8 cm × 4 cm retroperitoneal tumour, which eventually was diagnosed as PEComa of uncertain biological behaviour. Three years after the operation, the patient remains without any evidence of recurrence. A search was performed in the Medline and EMBASE databases for articles published between 1996 and 2018, and we identified 31 articles related to retroperitoneal and perinephric PEComas. We focused on sex, age, maximum dimension, histological and immunohistochemical characteristics of the tumour, follow-up and long-term outcome. Thirty-four retroperitoneal (including the present one) and ten perinephric PEComas were identified, carrying a malignant potential rate of 44% and 60%, respectively. Nearly half of the potentially malignant PEComas presented with or developed metastases during the course of the disease. CONCLUSION: Retroperitoneal PEComas are not as indolent as they are supposed to be. Radical surgical resection constitutes the treatment of choice for localized disease, while mammalian target of the rapamycin (mTOR) inhibitors constitute the most promising therapy for disseminated disease. The role of mTOR inhibitors as adjuvant or neoadjuvant therapies needs to be evaluated in the future.

Malignant proliferating trichilemmal tumour of the scalp. Report of a case and a short review of the literature.

BACKGROUND: Malignant proliferating trichilemmal tumour is a rare skin tumour that originates from the root sheath of the hair and usually arises in the sun-exposed areas of elderly women. It mimicks poorly differential squamous cell carcinoma ant its biological behaviour is unpredictable, because rarely can produce distant metastases. CASE REPORT: The Authors report on a case of a 54-year-old female patients who proceeded with a cystic lesion in the scalp. The lesion was removed with wide excision. The histological examination of the lesion revealed a malignant proliferating trichilemmal tumour. Two years after the excison the patients is free of disease. DISCUSSION: Only few cases (about seventy) of malignant proliferating trichilemmal tumours have been reported in the Bibliography, in some of the patients with malignancy, the tumour produces distant metastases. In the reported case, although the high risk of distant metastasis, two years after the tumour resection the patient is free of disease, It is also reported a short review of the literature. CONCLUSIONS: Malignant proliferating trichilemmal tumours are rare malignant lesions that affect mainly older women. The appropriate treatment includes wide resection and dose postoperative follow-up of the patient to facilitate the early diagnosis of distant metastases.

Fournier's gangrene. Immediate diagnosis and multimodality treatment is the comerstone for successful outcome.

BACKGROUND: Fournier's gangrene is a synergistic necrotic fasciitis or myositis of genitalia, perineum and abdominal wall. Several etiologic factors have been reported, microorganisms isolated and co-morbidity factors identified. Immediate and accurate diagnosis decreases mortality rate, which ranges from 3-67%. Aggressive resuscitation and surgical debridement consist the appropriate treatment. MATERIAL AND METHODS: Six cases of Fournier's gangrene treated the last ten years were reviewed. Three of them presented with mild infection while the other three with severe. The mean time interval between first symptoms and initial treatment was 2.1 days. The diagnosis was set immediately and the treatment included fluid-electrolyte resuscitation, aggressive surgical debridement, and broad-spectrum antibiotics administration. RESULTS: All patients survived. The defects healed by second intention in four patients and only in two patients specific plastic reconstructive techniques were required. Hyperbaric oxygenation administrated in one patient induced surprisingly the healing of the lesion. CONCLUSIONS: The Authors confirm that Fournier's gangrene is a rare and potentially fatal disease. Early suspicion, accurate diagnosis and multimodality treatment including aggressive surgical intervention are essential for successful outcome.