Endoscopic management of sinonasal hemangiopericytoma: case report and literature review

ABSTRACT Introduction: Hemangiopericytoma is a rare vascular tumor of the sinonasal region, associated with epistaxis and nasal obstruction as the main symptoms. When located in this region, it has special clinical characteristics that differentiate it from others. Case presentation: The following paper reports the case of a 43-year-old female patient presenting with right nasal obstruction and 6 months of evolution associated with mucopurulent rhinorrhea and recurrent right side epistaxis. Physical examination showed a right obstructive mass originating from the cribriform plate. Computed tomography of the paranasal sinuses revealed a complete blockage of the right nasal cavity by a homogeneous content, with soft tissue density and no evident contrast enhancement. The lesion extended superiorly to the cribriform plate but without intracranial or orbital extension. The patient was treated with endoscopic surgery and anatomopathological study revealed sinonasal hemangiopericytoma. The patient had complete remission and subsequent 3-year follow-up without recurrence. Conclusion: The recommended treatment for hemangiopericytoma is total surgical excision with free margins. The results are generally good and the risk of recurrence seems to be associated with incomplete tumor excision. A literature review is presented and its main characteristics are discussed.

RESUMEN Introducción: El hemangiopericitoma es un tumor vascular poco frecuente en la región nasosinusal que se asocia con epistaxis y obstrucción nasal como principales síntomas. Cuando se presenta en esta región, tiene características clínicas especiales que lo hace diferente de otras localizaciones. Presentación del caso: A continuación se presenta el caso de una paciente de 43 años de edad, con síntomas de obstrucción en fosa nasal derecha y 6 meses de evolución asociados con rinorrea mucopurulenta y epistaxis recurrente del lado derecho. El examen físico mostró una masa obstructiva en el lado derecho con origen en la placa cribriforme. Una tomografía computarizada de los senos paranasales reveló un bloqueo completo de la cavidad nasal derecha por un contenido homogéneo, con densidad de partes blandas, sin captación de contraste evidente. La lesión se extendía hacia el lado superior de la placa cribriforme, sin extensión intracraneal u orbitaria. La paciente fue tratada con cirugía endoscópica y el estudio anatomopatológico reveló un hemangiopericitoma sinonasal. La paciente tuvo remisión completa y se realizó seguimiento por 3 años sin recurrencia. Conclusión: El tratamiento recomendado para el hemangiopericitoma es la escisión quirúrgica total con márgenes libres, cuyos resultados son generalmente buenos. El riesgo de recurrencia parece estar asociado con una escisión tumoral incompleta. Se presenta una revisión de literatura, así como comentarios sobre sus características principales.

Nodular fasciitis on the zygomatic region: a rare presentation / Fasciite nodular na regiao zigomatica: uma apresentacao rara

Nodular fasciitis is a benign tumor, resulting from reactive proliferation composed of fibroblastic/myofibroblastic cells. Due to its rapid growth and high cellularity it may be mistaken for sarcoma. Despite the possibility of spontaneous regression, excision is the treatment of choice. A 24-year-old female patient presented with a nodule on the zygomatic region with 3 months of evolution. Excisional biopsy was performed. Histopathological examination associated with immunohistochemical markers HHF35, AML and Ki-67 allowed diagnostic confirmation. The main relevance of the case presented is its rare location, suggesting its inclusion among the differential diagnoses of tumor lesions on the face.

A fasciíte nodular é um tumor benigno, decorrente da proliferação reativa de células fibroblásticas ou miofibroblásticas. Pode ser confundida com os sarcomas, devido a seu rápido crescimento e rica celularidade. Apesar da possibilidade da involução espontânea, a excisão é o tratamento de escolha. É apresentada uma paciente de 24 anos, com um nódulo de 3 meses de evolução na região zigomática. Foi realizada biópsia excisional. O exame histopatológico associado à imuno-histoquímica com os marcadores HHF35, AML e Ki-67 permitiram a confirmação diagnóstica. A principal relevância do caso apresentado é sua rara localização, sugerindo a sua inclusão entre os diagnósticos diferenciais das lesões tumorais na face.

Case for diagnosis / Caso para diagnóstico

Infantile digital fibromatosis or Reye's tumor is a benign fibroproliferative tumor, the etiopathogenesis of which has yet to be fully clarified. It typically presents at birth or in the first year of life and is characterized by a firm, flesh colored or erythematous nodule or nodules located on the digits. These lesions tend to regress spontaneously.

A fibromatose digital infantil ou tumor de Reye é um tumor fibroso benigno de etiologia desconhecida, presente ao nascimento ou com aparecimento no primeiro ano de vida. Caracteriza-se por nódulos firmes, únicos ou múltiplos, normocrômicos ou eritematosos, localizados nas falanges, com tendência à regressão espontânea.

Neurofibroma subungueal solitário: relato de um caso inédito no sexo masculino / Solitary subungual neurofibroma: a previously unreported finding in a male patient

Neurofibroma é uma proliferação hamartomatosa de origem neuromesenquimal. Pode ser encontrado associado à neurofibromatose ou como tumor solitário, sendo sua apresentação subungueal solitária bastante rara. É mais frequente em pacientes do sexo feminino e a cirurgia é o tratamento de escolha. Relatase um caso de um paciente masculino, com lesão tumoral subungueal no pododáctilo, cuja biópsia e imuno-histoquímica foram compatíveis com neurofibroma. Até a presente data, menos de dez casos sobre neurofibromas subungueais sem associação com doença de Von Recklinhausen foram documentados, sendo este o primeiro caso relatado no Brasil e o único no sexo masculino no mundo.

A neurofibroma is a hamartomatous proliferation of neuromesenchymal origin. It may be found in combination with neurofibromatosis or in the form of a solitary tumor. Clinical presentation as a solitary subungual tumor is very rare. Neurofibroma is more common in females and surgery is the treatment of choice. The present paper reports the case of a male patient with a subungual tumor on his toe. Biopsy and immunohistochemistry findings were compatible with a neurofibroma. To date, fewer than ten cases of subungual neurofibromas unassociated with von Recklinghausen's disease have been documented, this being the first case to be reported in Brazil and the only report worldwide to have described this condition in a male patient.

Miofrioblastoma mamário em axila / Mammary myofibroblastoma in axila

Miofibroblastoma é um tumor benigno raro de mama, de origem mesenquimal. A localização em região axilar é ainda mais incomum. Tendo em vista a dificuldade em se estabelecer o diagnóstico diferencial baseado apenas nos achados clínicos e imaginológicos, a confirmação histopatológica é imprescindível no diagnóstico diferencial com tumor oculto de mama.

Myofibroblastoma is a rare and benign mesenchymal breast neoplasm. The axillary topography is further more uncommon. The histologic identification is important because the differencial diagnosis with malignant tumours of the breast. This report a case of mammary myofibroblastoma in axilla, wich was initially diagnosticated as an occult lobular carcinoma.

Hamartoma fibroso de la infancia: informe de un caso / Fibrous hamartoma of infancy: case report

Introducción: El hamartoma fibroso de la infancia es una proliferación benigna de los tejidos blandos, de presentación infrecuente; 91 % de los casos ocurre durante el primer año de edad y afecta más frecuentemente al sexo masculino. Se caracteriza por ser una lesión subcutánea de morfología característica con patrón de crecimiento organoide trifásico. Caso clínico: Lactante masculino de seis meses de edad, con tumor en la región plantar medial del pie izquierdo, el cual fue resecado completamente. En el estudio histopatológico se informó como hamartoma fibroso de la infancia. Conclusiones: Puede localizarse en cualquier sitio anatómico, aunque 5 a 10 % afecta las extremidades inferiores existen pocos casos informados en el pie. En biopsias pequeñas es importante su diagnóstico diferencial con otras lesiones fibroadiposas para el adecuado tratamiento. La resección quirúrgica amplia con márgenes libres de lesión confiere un buen pronóstico a los pacientes.

BACKGROUND: Fibrous hamartoma of infancy (FHI) is an infrequent benign proliferation of the soft tissues. Ninety one percent of cases occur during the first year of life. FHI is characterized as a subcutaneous lesion with characteristic morphology with a triphasic organoid growth pattern. CLINICAL CASE: We present the case of a 6-month-old male infant with a tumor in the medial plantar region of the left foot, which was completely withered. Histopathological study reported a fibrous hamartoma of infancy. CONCLUSIONS: Fibrous hamartoma of infancy most frequently affects males. It may be localized at any anatomic site, although 5-10% of cases affect the lower limbs. Few cases are reported in the foot. This is a lesion with a characteristic morphological pattern; however, in small biopsies, its differential diagnosis is important with other fibroadipose lesions for appropriate treatment. Ample surgical resection with lesion-free borders confers a good prognosis for these patients.

Tumor fibroso solitário na pele: relato de caso / Solitary fibrous tumor of the skin: case report

O tumor fibroso solitário (TFS) é uma neoplasia benigna muito rara, derivada de células mesenquimais. Há relatos de casos deste tumor em diversos sítios pleurais ou extrapleurais, sendo a região cutânea e subcutânea localizações ainda mais incomuns. O prognóstico, na maioria dos casos, é bom, mas o comportamento pode ser agressivo. O diagnóstico clínico diferencial é importante na prática diária do cirurgião plástico, pois não raramente aparecem lesões tumorais dos componentes do revestimento cutâneo, com características semelhantes ao TFS - consistência fibro elástica e não aderência a planos profundos - que devem ser bem conhecidas e diferenciadas de neoplasias malignas, para que o tratamento adequado seja instituído.

Solitary fibrous tumor (TFS) is a very rare benign tumor, derived from mesenchymal cells. There are reports of cases of this tumor in different pleural or extra-pleural’s sites, and the skin is even a more unusual spot. The prognosis in most cases is good, but the behavior in few cases can be aggressive. The differential diagnosis is important in the daily practice of plastic surgeons. There are several lesions well known with similar characteristics to TFS in skin layers: fibro elastic consistency, not adhered to deep planes. Those lesions should be differentiated from malignant neoplasms, so that proper treatment is instituted.

Frecuencia de tumores benignos durante el periodo de 2000-2006 en el Hospital General Dr. Manuel Gea González / Frequency of benign tumors at the Hospital General Dr. Manuel Gea Gonzalez: record review between 2000-2006

Antecedentes: Los tumores benignos de la piel se definen como el crecimiento autónomo de tejido sin relación con el patrón normal de crecimiento tisular, que persiste después del estímulo que le dio origen. Prácticamente todos los humanos presentan algún tumor benigno, pero la mayoría de las veces no se busca atención ya que no producen molestias. Son escasos los registros sobre la frecuencia de los tumores benignos. El objetivo de este estudio fue informar el número de tumores benignos en el Departamento de Dermatología de nuestro hospital. Material y métodos: Se realizó un estudio retrospectivo en el que se revisaron los registros de histopatología del periodo 2000 a 2006. Se registró año, número de biopsia, sexo, edad, topografía, diagnóstico clínico y diagnóstico histológico. Resultados: Se analizaron 9436 registros de biopsias, de las cuales 3765 fueron tumores benignos, de éstos se eliminaron 595 biopsias por falta de datos, para un total de 3170. Los tumores más frecuentes por grupo histopatológico en forma descendente fueron los tumores melanocíticos, quistes, tumores fibrosos, vasculares, de la epidermis, de tejido adiposo, del pelo, neurales, glandulares, sebáceos, de cartílago y hueso, y de músculo liso. Los tumores más frecuentes en general fueron nevos melanocíticos, quistes epidermoides, queratosis seborreicas, granulomas piógenos, lipomas y dermatofibromas. Conclusiones: Los tumores benignos más frecuentes fueron los melanocíticos constituidos por nevos de unión, intradérmicos y compuestos, y los quistes epidermoides. Esta serie proporciona información de los tumores benignos más comunes en un servicio dermatológico.

BACKGROUND: Benign skin neoplasms are defined as autonomous growing tissue unrelated to normal growing of the skin, that persist even after the originating stimulus dissapears. Almost all human beings have a certain number of benign cutaneous neoplasms and many never seek medical attention. There is a dearth of information regarding the frequency of these tumors. The aim of this study was to record the number of benign tumors studied at the Dermatology Department of a medical facility. MATERIAL AND METHODS: A retrospective study analyzed medical records between 2000 and 2006. We included year of admission, number of biopsies, sex, age, tumor location, histological and clinical diagnoses. RESULTS: We analyzed 9,436 biopsies of which 3,765 constituted benign neoplasms; 595 were not included and our total sample was 3,170 tumors. The most frequent tumors according to histopathological diagnoses in descending order were: melanocytic, cutaneous cysts, fibrous tumors, vascular tumors, epidermal tumors, fat tumors, tumors with hair differentiation, neural tumors, glandular tumors, tumors with sebaceous differentiation, cartilage and bone tumors, and smooth muscle tumors. The most common benign tumors were: Melanocytic nevi, epidermal cysts, seborrheic keratoses, pyogenic granulomas, lipomas and dermatofibromas. CONCLUSIONS: Melanocytes represented by melanocytic nevi (junctional, intradermic and compound) were the most frequent benign neoplasms, followed by epidermoid cysts. Our results illustrate the most common benign tumors observed in a dermatology department.