Carcinoma neuroendocrine de célula pequeña en la amígdala. Un caso clínico / Small Cell neuroendocrine carcinoma of the tonsil. A case report

Primary small neuroendocrine carcinoma (SNEC) of the tonsil it’s a rarity. We present the case of a 64 year-old Caucasian male patient referred to our hospital due to a neck mass. A fixed non-tender rock hard supraclavicular right mass and ipsilateral cervical lymphadenopathy was palpable. Biopsy was performed and showed poor differentiated neoplasm with features of SNEC. A fludeoxyglucose positron emission tomography (FDG-PET) scan showed intense uptake in the right tonsil. The patient underwent right tonsillectomy and immunochemistry was positive for characteristic features of small cell neuroendocrine carcinoma. Right tonsil SNEC was diagnosed and the patient was proposed to chemotherapy. (AU)

Histological Subtypes and Clinical Behavior Evaluation of Salivary Gland Tumors.

INTRODUCTION: Salivary gland tumors include a wide spectrum of histological subtypes and clinical behavior, which we aim to evaluate. MATERIAL AND METHODS: We performed a retrospective study of all salivary gland tumors diagnosed and treated at the Centro Hospitalar São João, Porto, between 2005 and 2015. Histological re- evaluation was performed in all cases and patient files were reviewed and both clinical and follow-up data were collected. Disease-free survival and overall survival were evaluated using Kaplan-Meier survival curves and compared using the Mantel-Cox log-rank test. The significance threshold was set at 0.05. RESULTS: We selected 295 cases, 150 males with a mean age at diagnosis of 50.4 (± 16.4) years. Primary benign epithelial tumors [n = 228 (77.3%)] were mostly pleomorphic adenomas [n = 148 (64.9%)] and Warthin tumors [n = 61 (26.8%)]. Primary malignant epithelial tumors [n = 43 (14.8%)] included mucoepidermoid [n = 16 (37.2%)], adenoid cystic [n = 6 (14.0%)] and acinic cell [n = 5 (11.6%)] carcinomas; 32 (74.4%) in parotid, 2 (4.6%) in submandibular and 9 (21%) in minor salivary glands. Primary epithelial tumors were more frequently malignant in minor (33.3%) than in major (13.9%) salivary glands. Local recurrence occurred in 30.2% and distant metastases in 25.6% tumors. The mean disease-free interval was 26 (± 37.5) months; most metastases were in lung and central nervous system. The 5 and 10 year disease-free survival rates were 63.4% and 50.1%, respectively; the 5 and 10 year disease-specific survival rates were 76.9% and 57.9%, respectively. Primary salivary tumors included also lymphomas [n = 8 (2.7%)] and soft tissue tumors [n = 5 (1.7%)]. Secondary tumors included metastases of carcinomas [n = 7 (2.4%)] and involvement by lymphoma [n = 1 (0.3%)]. DISCUSSION: Our results concerning age, gender, histological subtype, frequency and clinical behavior of salivary tumors concur with European studies. Divergence with Portuguese studies might be related with the inclusion criteria, clinical referral and time lag variations. CONCLUSION: Although uncommon, salivary gland tumors occur in a wide age range and include histological subtypes with diverse prognosis.

Introdução: Os tumores das glândulas salivares incluem um vasto espetro de subtipos histológicos e comportamento clínico que pretendemos avaliar. Material e Métodos: Realizou-se um estudo retrospetivo de todos os tumores das glândulas salivares diagnosticados e tratados no Centro Hospitalar São João, Porto, entre 2005 e 2015. Realizou-se reavaliação histológica de todos os casos. Os processos clínicos foram consultados e foram colhidos dados clínicos e de follow-up. A sobrevida livre de doença e a sobrevida global foram avaliadas usando as curvas de sobrevida de Kaplan-Meier e comparadas com o teste de log-rank Mantel-Cox. O limiar de significância foi estabelecido em 0,05. Resultados: Selecionaram-se 295 casos, 150 no género masculino, com idade média de 50,4 anos. Nos tumores epiteliais benignos [n = 228 (77,3%)] predominaram os adenomas pleomórficos [n = 148 (64,9%)] e os tumores de Warthin [n = 61 (26,8%)]. Os tumores epiteliais malignos primários [n = 43 (14,8%)] incluíram carcinomas mucoepidermoides [n = 16 (37,2%)], adenoide-císticos [n = 6 (14,0%)] e de células acinares [n = 5 (11,6%)]; 32 (74,4%) localizaram-se na parótida, dois (4,6%) na submandibular e nove (21%) nas glândulas salivares minor. Os tumores epiteliais primários foram mais frequentemente malignos nas glândulas salivares minor (33,3%) do que nas major (13,9%). Observou-se recidiva local em 30,3% e metástases em 25,6%. O intervalo médio livre de doença foi 26 meses. As metástases mais frequentes ocorreram no pulmão e no sistema nervoso central. A taxa de sobrevida livre de doença aos cinco e 10 anos foi 63,4% e 50,1%, respetivamente; a sobrevida aos cinco e 10 anos foi 76,9% e 57,9%, respetivamente. Nos tumores primários identificaram-se também linfomas [n = 8 (2,7%)] e tumores mesenquimatosos [n = 5 (1,7%)]; nos tumores secundários identificaram-se metástases de carcinoma [n = 7 (2,4%)] e envolvimento por linfoma [n = 1 (0,3%)]. Discussão: Estes resultados assemelham-se aos da literatura europeia, designadamente quanto à idade, género, subtipo histológico e comportamento clínico. As divergências com séries portuguesas podem relacionar-se com critérios de inclusão, enviesamentos de referenciação clínica e período dos estudos. Conclusão: Os tumores das glândulas salivares, embora raros, ocorrem numa faixa etária ampla, incluindo subtipos histológicos com prognósticos diferentes.

Pulmonary inflammatory myofibroblastic tumor: report of 2 cases with radiologic-pathologic correlation.

Inflammatory myofibroblastic tumor is a rare benign tumor that affects most commonly children and young adults. In the lung, it comprises less than 1% of all neoplasms. The authors describe the clinical, radiological, and pathologic features of 2 cases of incidentally discovered pulmonary inflammatory myofibroblastic tumors.

Small bowel intussusception in 2 adults caused by inflammatory polyps.

Inflammatory fibroid polyps are rare, benign pseudotumors of the gastrointestinal tract of unknown etiology, which may rarely present as bowel intussusception and obstruction. The authors describe the clinical, radiologic, and pathologic features of 2 patients with ileal inflammatory fibroid polyps presenting as small bowel intussusception.