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Solitary fibrous tumor (SFT) is a soft tissue tumor of mesenchymal origin involving, most commonly, the pleura. Intrapulmonary SFT is a slow-growing tumor that rarely reaches giant forms. SFTs are asymptomatic and often randomly discovered by routine chest X-rays. The diagnosis requires histopathological and immunohistochemical (IHC) examinations. Most of the SFTs are benign and present an indolent course. Larger tumors are more likely to be malignant and consequently associated with a worse prognosis. Despite having histopathological criteria for malignancy, the behavior of SFTs is challenging to predict. We report a case of giant intrapulmonary SFT of intermediate risk.

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ABSTRACT Solitary fibrous tumor (SFT) is a soft tissue tumor of mesenchymal origin involving, most commonly, the pleura. Intrapulmonary SFT is a slow-growing tumor that rarely reaches giant forms. SFTs are asymptomatic and often randomly discovered by routine chest X-rays. The diagnosis requires histopathological and immunohistochemical (IHC) examinations. Most of the SFTs are benign and present an indolent course. Larger tumors are more likely to be malignant and consequently associated with a worse prognosis. Despite having histopathological criteria for malignancy, the behavior of SFTs is challenging to predict. We report a case of giant intrapulmonary SFT of intermediate risk.

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RESUMEN Introducción: Los tumores fibrosos solitarios (TFS) son neoplasias raras de origen mesenquimal que, aunque generalmente se desarrollan en la pleura visceral, ocasionalmente se presentan en la cavidad intracraneal. Además, se caracterizan por altas tasas de metástasis y recurrencia. Caso clínico: Se presenta el caso de un paciente masculino de 59 años con cuadro de 3 meses de cefalea y bradipsiquia. La tomografía reveló una tumoración neoformativa que infiltra cavidad nasal, celdillas etmoidales, y fosa craneal anterior, comprometiendo lóbulo frontal izquierdo. El paciente fue sometido a dos craniectomías exploratorias donde se realizaron los diagnósticos sugestivos de neoplasia glial de alto grado y TFS. Para la precisión diagnóstica se realizó inmunohistoquímica que fue compatible con tumor fibroso solitario. Se analiza el caso centrándose particularmente en los aspectos histopatológicos, localización inusual de este tumor y sus manifestaciones clínicas variables.

ABSTRACT Introduction: Solitary fibrous tumors (SFTs) are rare mesenchymal neoplasms that, although typically develop in the visceral pleura, occasionally occur in the intracranial cavity. Furthermore, they are characterized by high rates of metastasis and recurrence. Case Report: We present the case of a 59-year-old male patient with a 3-month history of headache and bradyphrenia. Computed tomography revealed a neoformative tumor infiltrating the nasal cavity, ethmoid sinuses, and anterior cranial fossa, involving the left frontal lobe. The patient underwent two exploratory craniectomies, during which diagnoses suggestive of high-grade glial neoplasia and SFT were made. For precise diagnosis, immunohistochemistry was performed, which was consistent with solitary fibrous tumor. The case is analyzed, focusing particularly on histopathological aspects, the unusual location of this tumor, and its variable clinical manifestations.

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Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge.

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Solitary fibrous tumor (SFT) is a benign mesenchymal neoplasm originally described in pleura with a rare presentation in the oral cavity. Herein, we report a case of a 28-year-old male patient who presented an asymptomatic slow-growing mass in the anterior part of the tongue. Intraoral examination revealed a well-circumscribed mass covered by normal mucosa with a fibrous consistency. Due to non-specific clinical findings, the initial diagnostic hypotheses include benign submucosal neoplasms such as leiomyoma, neurofibroma, SFT, and others. An excisional biopsy was performed. Microscopically, the tumor was surrounded by a thick fibrous capsule; hypo and hypercellular areas were arranged in a storiform pattern with a stroma formed by collagen and abundant vascularization. Tumor cells showed immunopositivity for CD34 and STAT-6 and no expression of CD99, AML, S-100, and Ki-67. According to these findings, the diagnosis of SFT was established. After 24 months, the patient is asymptomatic and has no evidence of recurrence. Although oral involvement is rare, SFT should be included in the differential diagnosis of oral submucosal lesions.

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ABSTRACT Solitary fibrous tumor (SFT) is a benign mesenchymal neoplasm originally described in pleura with a rare presentation in the oral cavity. Herein, we report a case of a 28-year-old male patient who presented an asymptomatic slow-growing mass in the anterior part of the tongue. Intraoral examination revealed a well-circumscribed mass covered by normal mucosa with a fibrous consistency. Due to non-specific clinical findings, the initial diagnostic hypotheses include benign submucosal neoplasms such as leiomyoma, neurofibroma, SFT, and others. An excisional biopsy was performed. Microscopically, the tumor was surrounded by a thick fibrous capsule; hypo and hypercellular areas were arranged in a storiform pattern with a stroma formed by collagen and abundant vascularization. Tumor cells showed immunopositivity for CD34 and STAT-6 and no expression of CD99, AML, S-100, and Ki-67. According to these findings, the diagnosis of SFT was established. After 24 months, the patient is asymptomatic and has no evidence of recurrence. Although oral involvement is rare, SFT should be included in the differential diagnosis of oral submucosal lesions.

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ABSTRACT Solitary fibrous tumor (SFT) is a rare fibroblastic mesenchymal neoplasm with an estimated annual incidence of 0.35 per 100,000 individuals. Doege-Potter syndrome is a paraneoplastic syndrome related to solitary fibrous tumor clinically characterized by hypoglycemia, occurring in less than 5% of cases. Herein, we report a case of metastatic SFT associated with recurrent severe hypoglycemia. A 43-year-old male with a noncontributory medical history presented with a painless and progressive growing mass in the right thigh. The histological evaluation rendered the diagnosis of SFT, and tumor resection was performed. One year after the operation, on the oncological follow-up, he was admitted to the emergency unit, manifesting an early-morning seizure associated with a severe hypoglycemia. The laboratory findings of non-islet cell tumor hypoglycemia (NICTH) in the background of a relapsed metastatic solitary fibrous tumor were consistent with the diagnosis of Doege-Potter syndrome. Hepatic embolization associated with oral glucocorticoid was an efficient palliative treatment to control the hypoglycemic crisis and allow hospital discharge.

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Abstract A 58-year-old female patient presented with a single-digit clubbing on the second finger of her right hand two years previously. After investigation with imaging and incisional biopsy, superficial acral fibromyxoma was diagnosed. A brief review on single-digit clubbing and its causes is presented, focusing on superficial acral fibromyxoma.

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A 58-year-old female patient presented with a single-digit clubbing on the second finger of her right hand two years previously. After investigation with imaging and incisional biopsy, superficial acral fibromyxoma was diagnosed. A brief review on single-digit clubbing and its causes is presented, focusing on superficial acral fibromyxoma.

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Resumen Introducción : el tumor fibroso solitario de pleura (TFSP) es una patología poco frecuente, corresponde a menos del 5 % de los tumores primarios de pleura, siendo aún más raros los tumores mayores de 15 cm y aquellos en pacientes menores de 35 años, ya que el pico de incidencia es entre los 60 a 70 años con un solo caso reportado en Colombia. Presentación del caso : paciente femenina de 33 años, sin exposición a asbesto o cigarrillo. Consultó por cuadro de disnea de moderados esfuerzos y dolor dorsal derecho. La tomografía axial computarizada (TAC) contrastada de tórax evidenció una gran masa sólida de contornos lobulados que comprometía el 70 % del hemitórax derecho de origen extrapulmonar y que comprimía la aurícula derecha, la inmunohistoquímica de la biopsia percutánea clasificó la lesión como tumor fibroso solitario. Fue llevada a toracotomía posterolateral derecha extendida, donde se encontró un tumor dependiente de la pleura parietal derecha resecado en su totalidad. Histopatología e inmunohistoquímica del producto de resección quirúrgica, compatibles con tumor fibroso solitario de pleura, tamaño 30 x 23 x 16 cm sin características de malignidad. La evolución posoperatoria fue satisfactoria, con un egreso hospitalario temprano. Discusión : en una paciente joven, el TFSP gigante es inusual, posterior a su diagnóstico, y a pesar de tener algunas características que sugerían malignidad: tamaño y origen en la pleura parietal, la resección quirúrgica fue el tratamiento indicado para este tumor, con posterior confirmación histopatológica compatible con tumor benigno.

Abstract Introduction : The solitary fibrous tumor of the pleura (SFTP) is an uncommon pathology. It corresponds to less than 5 % of the primary tumors of the pleura. Even much rare is to find one solitary fibrous tumor, of more than 15 cm in patients younger than 35 years since the incidence peak is between 60 to 70 years. There has been only one case reported in Colombia. Case presentation : The subject of study was a 33-year-old female patient, no exposure to asbestos or cigarette. The chief complaints of the patient were mild dyspnea and right dorsal pain. Thoracic contrast-enhanced computerized tomography showed a large solid mass of lobulated contours that compromised 70 % of the right hemithorax, of extrapulmonary origin compressing the right atrium. Immunohistochemistry of the percutaneous biopsy demonstrated a solitary fibrous tumor. The patient underwent an extended right posterolateral thoracotomy. We found and completely resected a tumor hanging on the right parietal pleura. Histopathology and immunohistochemistry of the product of the surgical resection were compatible with a solitary fibrous tumor of the pleura, 30 x 23 x 16cm in size, and no malignancy. The evolution after surgery was satisfactory with an early hospital discharge. Discussion : In a young patient, a giant TFSP is unusual. After diagnosis and despite having some characteristics that suggested malignancy: its size and origin in the parietal pleura, surgical resection was the appropriate procedure for this tumor, with subsequent histopathological confirmation compatible with a benign tumor.

Resumo Introdução : o tumor fibroso solitário de pleura (TFSP) é uma patologia pouco frequente, corresponde a menos do 5 % dos tumores primários de pleura, sendo ainda mais estranhos os tumores maiores de 15 cm e aqueles em pacientes menores de 35 anos, pois o pico de incidência é entre os 60 a 70 anos com um só caso reportado na Colômbia. Apresentação de caso : paciente feminina de 33 anos, sem exposição a asbesto ou cigarro. Consultou por quadro de dispneia de moderados esforços e dor dorsal direita. A tomografia axial computadorizada (TAC) contrastada de tórax evidenciou uma grande massa sólida de contornos lobulados que comprometia o 70 % do hemitórax direito de origem extrapulmonar e que comprimia a aurícula direita, imuno-histoquímica da biopsia percutânea classificou a lesão como tumor fibroso solitário. Foi levada à toracotomia posterolateral direita estendida, encontrando um tumor dependente da pleura parietal direita ressecado em sua totalidade. Histopatologia e imuno-histoquímica do produto de ressecção cirúrgica, compatíveis com tumor fibroso solitário de pleura, tamanho 30 x 23 x 16cm sem características de malignidade. A evolução pós-cirúrgica foi satisfatória, conseguindo um egresso hospitalar precoce. Discussão : em uma paciente jovem, o TFSP gigante é inusual, posterior a seu diagnóstico e apesar de ter algumas características que sugeriam malignidade; tamanho e origem na pleura parietal, a ressecção cirúrgica foi o tratamento indicado para este tumor, com posterior confirmação hispatológica compatível com tumor benigno.

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ABSTRACT Solitary fibrous tumor (SFT) is a rare mesenchymal neoplasm, composed of monotonous spindle cells, intermingled with collagen fibers. Kidney location is sporadic, with few reports in literature. We present two cases of 40- and 48-year-old males, one with incidental detection of a peripheral mass; another with hilar lesion perceived following investigation of hematuria. Both revealed a spindle-cell proliferation, without mitosis and necrosis. Immunohistochemical studies: positivity for CD34, CD99 and B-cell lymphoma 2 (Bcl-2). No sign of disease were evident nine and five months after surgery, respectively. SFT is mostly benign, but can show malignant behavior. Morphologic and immunohistochemical characteristics are essential for diagnosis.

RESUMEN Tumor fibroso solitario (TFS) es una neoplasia mesenquimal infrecuente de células fusiformes monótonas, separadas por bandas de colágeno. Localización renal es poco habitual, con escasos casos descriptos. Reportamos dos casos de pacientes masculinos, de 40 e 48 anos, el uno con detección incidental de masaperiférica; el otro con lesión hiliar descubierta en la investigación de hematuria. Los dos revelaronproliferación fusocelular sin necrosis ni mitosis. Inmunohistoquímica: positividad para CD34, CD99 y linfoma de células B2 (Bcl-2). No hay evidencia de recidiva a los nueve y cinco meses de la cirugía, respectivamente. TFS es, por lo general, benigno, pero puede revelar comportamiento agresivo. Características morfológicas y inmunohistoquímicas son esencialespara el diagnóstico.

RESUMO Tumor fibroso solitário (TFS) é uma neoplasia mesenquimatosa rara composta por células fusiformes monótonas, intercaladas por fibras de colágeno. Localização renal é incomum, com escassos casos descritos. Relatamos dois casos do gênero masculino, com 40 e 48 anos, um com detecção incidental de massa periférica; outro com lesão hilar descoberta na sequência de investigação por hematúria. Ambos revelaram proliferação fusocelular sem mitoses nem necrose. Imuno-histoquímica: positividade para CD34, CD99 e linfoma de células B2 (Bcl-2). Não há evidência de recidiva nove e cinco meses após cirurgia, respetivamente. TFS é maioritariamente benigno, mas pode revelar comportamento agressivo. Características morfológicas e imuno-histoquímicas são essenciais para o diagnóstico.

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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.

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ABSTRACT Solitary fibrous tumor (SFT) is a rare mesenchymal spindle-cell neoplasm commonly found in the pleura; it is rare in the orbit and extremely rare in the lacrimal gland. We herein report a case of SFT of the lacrimal gland that mimicked a pleo morphic adenoma. We discuss the clinical, radiological, histopathological, and immunohistochemical findings that provided insight and rationale to accurately diagnose this case.

RESUMO Os autores relatam um caso de tumor fibroso solitário (TFS) de glândula lacrimal simulando um adenoma pleomórfico. O TFS é um raro tumor mesenquimal de células fusiformes, comumente encontrado na membrana pleural, raramente en contrado na órbita e extremamente raro na glândula lacrimal. Os autores discutem os achados clínicos, radiológicos, histológicos e imunohistoquímicos que são a chave para o correto diagnóstico desta rara entidade.

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Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that appears primarily in the pleura and rarely in intrapulmonary or endobronchial topography. The authors report the case of a 47-year-old woman who presented obstructive respiratory symptoms for 4 years. The chest computed tomography and bronchoscopy showed an obstructive polypoid lesion located between the trachea and the left main bronchus associated with distal atelectasis of the left lung. A resection of the lesion was performed and, macroscopically, the mass was oval, encapsulated, and firm, measuring 2.3 × 1.7 × 1.5 cm. Histology revealed low-grade mesenchymal spindle cell neoplasm, with alternating cellularity, myxoid areas, and mature adipose tissue outbreaks, as well as blood vessels with irregular walls. The immunohistochemical study was positive for CD34, CD99, and BCL2. The diagnosis was SFT in an unusual topography. The patient's symptoms remitted after tumor excision, and no systemic problems were evident. SFTs primarily affect adults and often follow a benign course; however, their behavior is unpredictable. The presence of necrosis and mitotic activity may portend a poor prognosis. Endobronchial SFTs are rare but should be evaluated and monitored similar to SFTs at other sites, with a long-term follow-up

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Solitary fibrous tumor (SFT) is a mesenchymal neoplasm that appears primarily in the pleura and rarely in intrapulmonary or endobronchial topography. The authors report the case of a 47-year-old woman who presented obstructive respiratory symptoms for 4 years. The chest computed tomography and bronchoscopy showed an obstructive polypoid lesion located between the trachea and the left main bronchus associated with distal atelectasis of the left lung. A resection of the lesion was performed and, macroscopically, the mass was oval, encapsulated, and firm, measuring 2.3 × 1.7 × 1.5 cm. Histology revealed low-grade mesenchymal spindle cell neoplasm, with alternating cellularity, myxoid areas, and mature adipose tissue outbreaks, as well as blood vessels with irregular walls. The immunohistochemical study was positive for CD34, CD99, and BCL2. The diagnosis was SFT in an unusual topography. The patient's symptoms remitted after tumor excision, and no systemic problems were evident. SFTs primarily affect adults and often follow a benign course; however, their behavior is unpredictable. The presence of necrosis and mitotic activity may portend a poor prognosis. Endobronchial SFTs are rare but should be evaluated and monitored similar to SFTs at other sites, with a long-term follow-up.

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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.

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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.

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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.

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