Intra-abdominal follicular dendritic cell sarcoma with marked pleomorphic features and aberrant expression of neuroendocrine markers: report of a case with immunohistochemical analysis.

Follicular dendritic cell sarcoma (FDCS) is a very rare malignant tumor arising most frequently in lymph nodes with only few reports of extranodal locations. We report the case of a 35-year-old man with a large retroperitoneal mass. Histologically the tumor was composed of highly pleomorphic cells exhibiting some uncommon features such as an epithelioid appearance, cystic spaces, and multinucleated cells with morphologic features of emperipolesis. Immunohistochemically the neoplastic cells were immunoreactive for CD21, CD23 and CD35. A previously unreported expression of neuroendocrine markers (Synaptophisyn and Neuron-Specific-Enolase) was present. Ultrastructurally no neuroendocrine secretory granules were detected. FDCS can mimic a wide variety of other malignant tumors, and a correct diagnosis requires exclusion of other neoplasms and immunohistochemical confirmation.

Tumor do estroma gastrintestinal: achados clínicos, radiológicos e anatomopatológicos / Gastrointestinal stromal tumor: clinical, radiologic and pathologic features

OBJETIVO: Investigar e descrever os achados clínicos, radiológicos e anatomopatológicos dos tumores do estroma gastrintestinal. MATERIAIS E MÉTODOS: De dezembro de 2000 a março de 2006, 16 pacientes foram operados por tumores do estroma gastrintestinal em nossa instituição. As variáveis analisadas foram sexo e idade dos pacientes, sinais e sintomas na consulta inicial, localização e tamanho do tumor, achados radiológicos, características anatomopatológicas e a ocorrência de metástases. RESULTADOS: A população em estudo constou de nove homens e sete mulheres. Os locais de origem dos tumores primários foram o estômago (n = 5), o reto (n = 4), o intestino delgado (n = 3), o mesentério (n = 3) e o cólon sigmóide (n = 1). Tomografia computadorizada foi o principal método radiológico empregado. Massa circunscrita, de contornos lobulados e que sofre realce heterogêneo pelo meio de contraste foi o principal achado por imagem. Em nosso estudo, nove pacientes (56 por cento dos casos) apresentaram metástases ao diagnóstico ou recorrência do tumor num período médio de dois anos e oito meses. CONCLUSÃO: O tumor do estroma gastrintestinal acomete adultos de meia-idade e idosos e deve ser lembrado no diagnóstico diferencial das massas abdominais. Diagnóstico precoce, tratamento correto e acompanhamento rigoroso são fundamentais, pois, como demonstrado em nosso trabalho, essas neoplasias apresentam alta tendência à malignidade.

OBJECTIVE: To investigate and describe clinical, radiologic and pathologic findings of gastrointestinal stromal tumors. MATERIALS AND METHODS: In the period between December 2000 and March 2006, 16 patients were submitted to surgery for gastrointestinal stromal tumors in our institution. The following variables were taken into consideration: sex and age, signs and symptoms at presentation, tumor site and size, radiological and pathological features, and presence of metastasis. RESULTS: The study population was constituted by nine men and seven women. The primary tumor sites of origin were: stomach (n = 5), rectum (n = 4), small bowel (n = 3), mesentery (n = 3), and colon (n = 1). Mean primary tumor size was 9 cm. Computed tomography was the main radiological method utilized. Circumscribed, lobulated and heterogeneously contrast-enhanced mass was the main image finding. Metastasis was found in nine patients (56 percent of cases) at presentation or tumor recurrence was observed during the follow-up period (mean = 32 months). CONCLUSION: Gastrointestinal stromal tumor occurs in middle-age adults and the elderly, and must be taken into consideration as differential diagnosis for abdominal masses. Early diagnosis, adequate therapy, and rigorous follow-up are essential, considering the high probability of malignancy of these neoplasms as demonstrated by the present study.

Description of a neural sheath tumor of the trigeminal nerve: immunohistochemical and electron microscopy study

CONTEXT: Malignant neural sheath tumors of the trigeminal nerve affecting the nasal cavity and the paranasal sinuses are extremely rare. With conventional optical microscopy, their identification is difficult, and it is necessary to confirm them by means of electron microscopy and immunohistochemical techniques. CASE REPORT: The patient was a 41-year-old woman with a ten-month progressive history of pain followed by painful edema in the left facial region, and with symptoms of bleeding, secretion and nasal obstruction. Studies with imaging methods suggested the presence of an expansive process in the left nasal and paranasal cavities. In the biopsy, the histopathological findings from optical microscopy were suggestive of a tumor of neural origin in the trigeminal nerve. Immunohistochemical and electron microscopy studies confirmed that it was a malignant tumor of the neural sheath of the trigeminal nerve. We describe the clinical, radiological, and histological features of this tumor and review the literature.

CONTEXTO: O tumor maligno da bainha neural do trigêmeo que acomete a cavidade nasal e os seios paranasais é extremamente raro. A microscopia óptica habitual sua identificação é difícil, sendo necessária a confirmação através de microscopia eletrônica e de técnicas imunoistoquímicas. RELATO DE CASO: A paciente era uma mulher de 41 anos com história de 10 meses de progressiva dor seguida de edema álgico em região facial à esquerda, e de sintomas de sangramento, secreção e obstrução nasal. Estudos com métodos de imagem sugeriam a presença de processo expansivo em cavidades nasais e paranasais à esquerda. A biopsia, os achados histopatológicos à microscopia óptica foram sugestivos de tumor de origem neural no trigêmeo. Estudos imunoistoquímico e por microscopia eletrônica confirmaram se tratar de neoplasia maligna da bainha neural do trigêmeo. Descrevemos as características clínicas, radiológicas e histológicas deste tumor, e revisamos a literatura.

Description of a neural sheath tumor of the trigeminal nerve: immunohistochemical and electron microscopy study.

CONTEXT: Malignant neural sheath tumors of the trigeminal nerve affecting the nasal cavity and the paranasal sinuses are extremely rare. With conventional optical microscopy, their identification is difficult, and it is necessary to confirm them by means of electron microscopy and immunohistochemical techniques. CASE REPORT: The patient was a 41-year-old woman with a ten-month progressive history of pain followed by painful edema in the left facial region, and with symptoms of bleeding, secretion and nasal obstruction. Studies with imaging methods suggested the presence of an expansive process in the left nasal and paranasal cavities. In the biopsy, the histopathological findings from optical microscopy were suggestive of a tumor of neural origin in the trigeminal nerve. Immunohistochemical and electron microscopy studies confirmed that it was a malignant tumor of the neural sheath of the trigeminal nerve. We describe the clinical, radiological, and histological features of this tumor and review the literature.

[Gastrointestinal stromal sarcoma. Report of a case and review of the literature]. / Sarcoma del estroma gastrointestinal. Presentacion de un caso y revision de la literatura.

The present report describes a case of a 27 year old patient with epigastric pain of moderate intensity, accompanied by hiporexia, weight loss and melena who was hospitalized in August 1.994 after 17 days of these signs and symptoms. Colonoscopy revelead a 6 cms polypoid, ulcerated lesion in the hepatic angle of the colon. At surgery, a 5 x 5 cms tumoral lesional 1.5 cms from the Treitz adhered to the small bowel was found. The gross findings of the specimen revealed an exophitic ulcerated tumor that measured 10 x 10 x 9 cms. Satellite nodules up to 2 cms in diameter were seen in contact with the large bowel mucosae. Light microscopy, immunohistochemical and ultrastructural studies, indicated a diagnosis of PLEXOSARCOMA.