Histiocitoma fibroso maligno: estudio hisotpatologico e inmunohistoquímico / Malignant Fibrous Histiocytoma: Histopathological and Immunohistochemical Study

El histiocitoma fibroso maligno (MFH) es el tumor de tejido blando más común en adultos. Generalmente se considera que surge de las células mesenquimales primitivas que muestran diferenciación histiocítica y fibroblástica parcial. Las observaciones inmunohistoquímicas sugieren que la expresión de marcadores del músculo liso en el llamado MFH es el resultado de la diferenciación miofibroblástica. El presente estudio tiene como objetivo correlacionarse entre el subtipo histipatológico y los parámetros clínicos, calificar los casos de MFH dependiendo de los criterios histopatológicos para la clasificación, y examinar los casos inmunohistoquímicamente para la diferenciación miofibroblástica utilizando marcadores musculares lisos en casos de MFH como una ayuda para un diagnóstico preciso para un diagnóstico preciso. . Este estudio incluye 26 muestras de tejidos blandos diagnosticados como MFH recolectados de laboratorios histopatológicos privados y gubernamentales en Basrah durante el período de enero de 2000 a octubre de 2005. 4 casos adicionales (un leiomioma, dos fibromas y un fibrosarcoma se tomaron como control positivo y negativo. Los casos de MFH (77%) estaban en el grupo de edad de 45 a 60 años. La edad media fue de 53.5 años con una relación hombre / mujer de 1.3: 1. Diecinueve casos (73%) se ubicaron en las extremidades principalmente en las extremidades inferiores. Diecisiete años. Los casos (65.4%) fueron primarios. Veintidós (84.8%) eran de subtipo pleomórfico, dos eran mixoides y 2 eran inflamatorios. Todos los casos recurrentes se consideraban como el Grado III, de los diecisiete casos principales eran de grado III, por lo que veinte y veinte Tres casos (88.5%) fueron de grado III, los 3 casos restantes fueron de grado II. No se registró tumor de grado I. La mayoría de los casos de subtipo pleomórficos (95.5%) fueron de grado III. Ambos casos de subtipo mixoide fueron de grado Ii. Desmin expr Se encontró la en 3 casos (11.5%), ninguno para la actina del músculo liso o la proteína S-100. No hubo correlación entre la expresión de desmin y el sitio tumoral, subtipo o grado, así como, con la edad y el sexo de los pacientes. Se encontró una fuerte asociación entre la expresión de desmin y los tumores recurrentes del 33,3%

Malignant fibrous histiocytoma (MFH) is the most common soft tissue tumor in adult. It is generally regarded as arising from primitive mesenchymal cells that show partial histiocytic and fibroblastic differentiation. Immunohistochemical observations suggest that the expression of smooth muscle markers in the so called MFH is a result of myofibroblastic differentiation. The present study is aimed to correlate between histipathological subtype and clinical parameters, to grade the MFH cases depending on the histopathological criteria for grading, and to examine the cases immunohistochemically for myofibroblastic differentiation using smooth muscle markers in cases of MFH as an aid for accurate diagnosis. This study including 26 soft tissue specimens diagnosed as MFH collected from private and governmental histopathological laboratories in Basrah during the period from January 2000 to October 2005. Additional 4 cases (one leiomyoma, two fibromas and one fibrosarcoma were taken as control positive and negative. Twenty cases of MFH (77%) were in the age group 45-60 years. The mean age was 53.5 year with male to female ratio of 1.3: 1. Nineteen cases (73%) were located in the extremities mainly the lower limbs. Seventeen cases (65.4%) were primary. Twenty two (84.8%) were of pleomorphic subtype, two were myxoid and 2 were inflammatory. All the recurrent cases were regarded as grade III, from the seventeen primary cases fourteen were of grade III, so twenty three cases (88.5%) were of grade III, the remaining 3 cases were of grade II. No grade I tumor was recorded. The majority of pleomorphic subtype cases (95.5%) were of grade III. Both cases of myxoid subtype were of grade II. Desmin expression was found in only 3 cases (11.5%), none for smooth muscle actin or S-100 protein. There was no correlation between desmin expression and tumor site, subtype or grade, as well as, with age and sex of the patients. A strong association between desmin expression and recurrent tumors 33.3% was found.

Mixofibrosarcoma mandibular de bajo grado en un adulto / Low grade mandibular myxofibrosarcoma in an adult

Se describe el caso clínico de un paciente de 61 años de edad, con antecedente de buena salud, atendido en el St. Paul's Milennium Medical Hospital de Etiopía por presentar, desde hacía 2 años, una lesión en la encía marginal, con abombamiento de ambas corticales, que se extendía del primer premolar inferior derecho al izquierdo, por lo cual se decidió ingresarlo en el Servicio de Cirugía Maxilofacial para su estudio y tratamiento. Se trataba de un tumor poco frecuente y de gran tamaño, con amplia diseminación ganglionar hacia los niveles IV y V de ambas partes del cuello, de manera que se realizó el procedimiento quirúrgico. La evolución fue favorable en el posoperatorio inmediato y egresó de la institución 7 días después, pero continuó con radioterapia. Y seguimiento por consulta externa durante 24 meses después.

The clinical case of a 61-year-old patient, with a history of good health, treated at St. Paul's Milennium Medical Hospital in Ethiopia for presenting, for 2 years, a lesion in the marginal gum, with bulging of the gums, is described. both cortices, which extended from the lower right to the left first premolar, for which it was decided to admit him to the Maxillofacial Surgery Service for study and treatment. It was a rare and large tumor, with extensive lymph node dissemination to levels IV and V in both parts of the neck, so the surgical procedure was performed. The evolution was favorable in the immediate postoperative period and he was discharged from the institution 7 days later, but continued with radiotherapy. And follow-up by outpatient service for 24 months afterwards.

A case of pseudomyogenic hemangioendothelioma misdiagnosed as low-grade malignant fibrous histiocytoma and review of literature / 中南大学学报(医学版)

Pseudomyogenic hemangioendothelioma (PHE) is a rare angiogenic tumor. Histologically, the morphological characteristics of neoplastic vessels and endothelial differentiation are not obvious, and it is easy to be confused with epithelioid sarcoma, epithelioid hemangioendothelioma and myogenic tumor. PHE usually occurs in arms and legs in young people and has a significant male predominance. The tumor has a predilection for the distal extremities and its typical manifestation is multiple center invasion of a single limb, which can involve all layers of skin and subcutaneous tissues,and is often accompanied by abvious pain. Histologically, PHE is characterized by infiltrative growth of tumor. Most tumor lesions are composed of sheets and loose fascicles of plump spindle or epithelioid cells within a background of variably prominent inflammatory infiltration, which was commonly composed of neutrophils. Some cells may resemble rhabdomyoblasts, and nuclear atypia and mitosis were rare. The tumor cells generally expressed positive cytokeratin (CK), ETS-related gene (ERG), Friend leukemia virus integration 1 (FLI1) and integrase interactor 1(INI1). In some cases, the tumor cells expressed CD31. A case of a young woman was reported in this paper, who presented with a subcutaneous mass with severe pain and was chronologically misdiagnosed with herpes zoster, low-grade malignant fibrous histiocytoma and epithelioid hemangioendothelioma. In this study, the clinical and pathological features, differential diagnosis and the latest progress in therapy of PHE were analyzed based on relevant literature.

Fibrohistiocitoma maligno de face: uma apresentação rara / Malignant fibrohistiocytoma of face: a rare presentation

Contexto: O fibrohistiocitoma maligno é um sarcoma de tecidos moles muito agressivo, com rara apresentação limitada à pele e tecido subcutâneo em face. O diagnóstico é anatomopatológico com auxílio da imuno-histoquímica. Descrição do caso: Este artigo relata o caso de um paciente com diagnóstico de fibrohistiocitoma maligno restrito à face com boa resposta terapêutica após exérese cirúrgica. Discussão: Tendo em vista a raridade dessa afecção, dificuldade diagnóstica devido ao quadro inespecífico e com rápida evolução, é importante lembrar desse possível diagnóstico e atuar precocemente. Conclusões: O diagnóstico precoce interfere de forma significativa na evolução do quadro, sendo necessária a manutenção do acompanhamento oncológico e dermatológico com o intuito de detectar precocemente recidivas locais e metástases a distância.

Tumor Fibro-histiocítico Plexiforme de Vulva: Relato de Caso / Vulva Plexiform Fibrohistiocytic Tumor: Case Report / Tumor Fibrohistiocítico Plexiforme de la Vulva: Reporte de Caso

Introdução: O tumor fibro-histiocítico plexiforme é uma condição clínica rara, com cerca de 150 casos descritos na literatura. Relato do caso: Paciente, 23 anos, sexo feminino, portadora de lesão nodular em região vulvar sem características de malignidade em exames de ultrassonografia. Realizou acompanhamento clínico, tendo apresentado aumento do tamanho da lesão inicial, sendo optado então por realização de ressecção cirúrgica da lesão. Em estudo histopatológico, evidenciou-se lesão compatível com tumor fibro-histiocítico plexiforme, com margens cirúrgicas livres. No seguimento, a paciente foi encaminhada para avaliação oncológica com a intenção de realizar exames complementares de rastreio e descartar diagnósticos morfologicamente semelhantes. Diante dos exames complementares confirmando o diagnóstico inicial e sem evidência de doença metastática após excisão cirúrgica completa com margens livres, foi iniciado seguimento clínico. Conclusão: Relatar o caso é de extrema importância para divulgar a apresentação clínica, etapas diagnósticas, tratamento proposto e compartilhar informações acerca de evolução clínica apresentada.

Introduction: Plexiform fibrohistiocytic tumor is a rare clinical condition, with about 150 cases described in the literature. Case report: 23-years-old, female patient with nodular lesion in vulva without malignancy characteristics on ultrasound image. She underwent clinical follow-up and after an increase in the size of the lesion, surgical resection was opted. Histopathological findings suggested plexiform fibrohistiocytic tumor, with free surgical margins. In follow up, she was referred to oncological evaluation for screening complementary exams and rule out morphologically similar diagnoses. After the complementary exams confirming the initial diagnosis of plexiform fibrohistiocytic tumor and without evidence of metastatic disease after complete surgical excision with free margins, the patient is in clinical follow-up. Conclusion: Reporting the case is extremely important to disseminate the clinical presentation, diagnostic steps, proposed treatment and share information about the clinical evolution presented.

Introducción: El tumor fibrohistiocítico plexiforme es una condición clínica poco frecuente, con unos 150 casos descritos en la literatura. Reporte del caso: Paciente de 23, sexo feminino, años con lesión nodular en una región vulvar sin características malignas en los exámenes de ultrasonido. Se sometió a un seguimiento clínico, habiendo presentado un aumento en el tamaño de la lesión inicial, y se decidió realizar la resección quirúrgica de la lesión. En un estudio histopatológico, se evidenció una lesión compatible con tumor fibrohistiocítico plexiforme con márgenes quirúrgicos libres. Fue referida para la evaluación oncológica, realizó pruebas complementarias para el cribado y para descartar diagnósticos morfológicamente similares. En vista de las pruebas complementarias que confirman el diagnóstico inicial y sin evidencia de enfermedad metastásica después de una escisión quirúrgica completa con márgenes libres, se inició el seguimiento clínico. Conclusión: Informar del caso es extremadamente importante para difundir la presentación clínica, etapas diagnósticas, tratamiento propuesto y compartir información sobre la evolución clínica presentada.

Rapidly progressive malignant fibrous histiocytoma of right atrium: a rare case report

Abstract We are going to present a case of malignant fibrous histiocytoma in the right atrium, which is a very rare entity. The patient had a right atrial mass, which prolapsed through the tricuspid valve into the right ventricle, causing functional tricuspid valve stenosis. The tumor was completely resected and the patient had an uneventful postoperative period. Histopathological examination reported malignant fibrous histiocytoma. The patient presented to the emergency department five weeks after discharge with dyspnea and palpitation. Echocardiography and magnetic resonance imaging revealed recurrent right atrial tumor mass. His clinical status has worsened, with syncope and acute renal failure. On the repeated echocardiography, suspected tumor recurrence was observed in left atrium, which probably caused systemic embolization. Considering the aggressive nature of the tumor and systemic involvement, our Heart Council decided to provide palliative treatment by nonsurgical management. His status deteriorated for the next few days and the patient succumbed to a cardiac arrest on the 4th day.

Fibrohistiocitoma maligno gigante de face: tratamento reparador microcirúrgico utilizando retalho miocutâneo transverso do reto do abdome - Relato de caso / Giant malignant fibrous histiocytoma of the face: case report of microsurgical repair using a transverse rectus abdominis myocutaneous flap

Introdução: A ressecção de tumores invasivos de cabeça e pescoço pode resultar em defeitos extensos e complexos exigindo reparação imediata. Uma das opções de reparação é a transferência, utilizando técnica de microcirurgia vascular, do retalho musculocutâneo do reto abdominal pediculado nos vasos epigástricos inferiores profundos (TRAM). O presente estudo tem como objetivo registrar um procedimento utilizado no tratamento reparador microcirúrgico de fibrohistiocitoma maligno gigante de face com retalho TRAM. Relato de Caso: Paciente procurou atendimento médico devido a lesão tumoral gigante em hemiface direita. Foi realizada a tomografia computadorizada do crânio revelando volumoso processo expansivo de aspecto vegetante com limites mal definidos. Após os procedimentos básicos no pré-operatório, realizou-se a exérese do tumor que acometia músculos masseter e temporal direito, glândula parótida, assoalho orbitário à direita e osso malar. Posteriormente, retirou-se o retalho microcirúrgico do músculo reto do abdome em conjunto com a artéria epigástrica inferior profunda através de incisão cirúrgica da área hipogástrica. Em seguida, dissecção da artéria e veia facial utilizando microscópio e anastomoses venosa e arterial. Quanto à evolução retalho apresentou-se íntegro, com boa perfusão, sem sinais de infecção. Conclusões: A reconstrução facial microcirúrgica oferece liberdade ao cirurgião de cabeça e pescoço para realizar grandes ressecções tumorais.

Introduction: The resection of invasive tumors of the head and neck can result in extensive and complex defects requiring immediate repair. One repair option is the transfer of a transverse rectus abdominis myocutaneous (TRAM) flap pedicled on deep inferior epigastric vessels using vascular microsurgery. This study aimed to register a procedure used in the microsurgical treatment of giant malignant fibrous histiocytoma of the face using a TRAM flap. Case Report: A male patient sought medical care for a giant tumoral lesion in the right hemiface. Computed tomography of the skull revealed a voluminous expansive process of vegetating aspect with poorly defined borders. The excision of the tumor affected the right masseter and temporalis muscles, parotid gland, and right orbital and malar bones. Subsequently, microsurgical withdrawal of the TRAM flap was performed with the deep inferior epigastric artery through a surgical incision in the hypogastric area. Dissection of the facial artery and vein under microscopy and venous and arterial anastomoses followed. The flap was intact with good perfusion and no signs of infection. Conclusions: Microsurgical facial reconstruction allows head and neck surgeons to resect large tumors.

Clinical Outcomes of Surgical Treatment for Primary Chest Wall Soft Tissue Sarcoma / 대한흉부외과학회지

BACKGROUND: This study investigated the clinical outcomes of surgical treatment of primary chest wall soft tissue sarcoma (CW-STS). METHODS: Thirty-one patients who underwent surgery for CW-STS between 2000 and 2015 were retrospectively reviewed. The disease-free and overall survival rates were estimated using the Kaplan-Meier method, and prognostic factors were analyzed using a Cox proportional hazards model. RESULTS: The median follow-up duration was 65.6 months. The most common histologic type of tumor was malignant fibrous histiocytoma (29%). The resection extended to the soft tissue in 14 patients, while it reached full thickness in 17 patients. Complete resection was achieved in 27 patients (87.1%). There were 5 cases of local recurrence, 3 cases of distant metastasis, and 5 cases of combined recurrence. The 5-year disease-free rate was 49%. Univariate analysis indicated that incomplete resection (p<0.001) and stage (p=0.062) were possible risk factors for recurrence. Multivariate analysis determined that incomplete resection (p=0.013) and stage (p=0.05) were significantly associated with recurrence. The overall 5- and 10-year survival rates were 86.8% and 64.3%, respectively. No prognostic factor for survival was identified. CONCLUSION: Long-term primary CW-STS surgery outcomes were found to be favorable. Incomplete microscopic resection and stage were risk factors for recurrence.

Long-Term Survival after Wide Resection of Malignant Fibrous Histiocytoma of the Chest Wall / 대한흉부외과학회지

Primary malignant fibrous histiocytoma (MFH) of the chest wall is extremely rare and is characterized by aggressive features, including a high incidence of local recurrence and distant metastasis. Surgical resection of the chest wall is the primary modality of management. However, surgical treatment is not generally recommended in patients with evidence of distant metastasis. Here, we present a case of chest wall MFH along with a schwannoma mimicking distant metastasis in the right upper arm. The patient was treated by radical en bloc resection and survived for more than 9 years without recurrence.

Analysis of the Clinical Characteristics of Cutaneous Malignant Tumors: 11-year Single-center Study in Daejeon City and Chungnam Province / 대한피부과학회지

BACKGROUND: The occurrence of cutaneous malignant tumors has been increasing worldwide due to changes in various environmental factors. OBJECTIVE: Our study aimed to analyze the overall tendency of clinical characteristics in single-center patients with cutaneous malignant tumors according to sex, age, duration, size, anatomic site, treatment, and concomitant diseases. METHODS: We retrospectively reviewed the medical records and clinical photographs of 319 patients diagnosed with cutaneous malignant tumors (basal cell carcinoma, squamous cell carcinoma, melanoma, lymphoma, Paget's disease, metastatic skin cancer, mycosis fungoides, angiosarcoma, dermatofibrosarcoma protuberans, Kaposi sarcoma, malignant fibrous histiocytoma, Merkel cell carcinoma) and 109 patients diagnosed with premalignant tumors (Bowen's disease) between January 2007 and January 2017. RESULTS: The average annual incidence of malignant cutaneous tumors was 2.4%. In total, 158 males (36.9%) and 270 females (63.1%) were included with a mean age of onset of 66 years. Among the malignant tumors, basal cell carcinoma (30.8%) was the most common, while the incidences of squamous cell carcinoma (18.9%), melanoma (6.3%), and lymphoma (4.4%) were also high. The predominantly involved anatomic sites were the face (67.3%), trunk (11.5%), and legs (10.9%). Most cases involved solitarily existing tumors (77.3%), and pruritus (21.7%) was the most common accompanying symptom. Among the 69.6% of patients who received treatment for their tumors, the most common method was surgical removal. No significant increase in concomitant diseases or environmental factors was detected. CONCLUSION: Our study is meaningful as an overall and recent review of various types of cutaneous malignancies that provides preliminary data for further large-scale nationwide studies.

Ultrasound and MRI features of malignant fibrous histiocytoma of soft tissue / 中国骨伤

OBJECTIVE@#To investigate ultrasound and MRI features of malignant fibrous histiocytoma (MFH) of soft tissue.@*METHODS@#Ultrasound, MRI images and pathological data of 12 patients with malignant fibrous histiocytoma in soft tissue confirmed by operation and pathology were analyzed from January 2012 to August 2018, inlcuding 7 males and 5 females, aged from 36 to 69 years old with an average age of 53 years old; the courses of disease ranged from 4 to 49 months with an average of 28 months. Clinical manifestations were soft tissue masses and pain in the affected limbs. Ultrasound, MRI and contrast-enhanced examination were performed before operation. The lesions, morphology, echo/signal characteristics, color flow signals and enhancement features were observed and compared with pathology.@*RESULTS@#In 12 patients with MFH, 9 patients were primary lesions and 3 patients were recurrent lesions after operation. There were 7 cases of bilateral thighs, 2 cases of calves, 1 case of upper arm, 1 case of buttocks and 1 case of posterior peritoneum. The size ranged from 5.1 to 17.1 cm with an average of 8.7 cm. Ultrasound feature showed lobulated or agglomerate, and focused on low echo; 5 cases had capsule and with clear border; 7 cases were unclear boundary with surrounding tissues; and 6 cases with irregular echo-free. The blood flow signals were around the CDFI, and the internal blood flow signals were different. MRI feature showed lobulated, agglomerate or irregular shape, T1WI showed slightly lower signal or equal signal, T2WI showed high signal and DWI signal increased. Six patients manifested mixed signal inside, 7 patients manifested low signal separation inside, 5 patients with false envelope, and 9 patients manifested infiltration and growth with peripheral edema. T1WI showed uneven strengthening after enhancement. Immunohistochemical expression of Vim, CD68 were positive.@*CONCLUSIONS@#The age, location and imaging features of soft tissue MFH are characteristic. The diagnosis of MFH should be considered when irregular mass occurred in soft tissues of limbs at middle-aged and old people. Echo and signal are homogeneous or mixed. Separation, necrosis and cystic degeneration could be seen in the mass. When the blood flow signals are abundant and solid components are obviously enhanced, the diagnosis of MFH should be considered.

Primary Undifferentiated Pleomorphic Sarcoma of the Colon Mesentery

An undifferentiated pleomorphic sarcoma (UPS), also known as a malignant fibrous histiocytoma in the past, commonly involves the soft tissue of the extremities and the retroperitoneum. However, a primary UPS of the colon mesentery is very rare. A 69-year-old male patient visited our outpatient department for treatment of an enlarged, palpable mass in the right lower quadrant (RLQ). Computed tomography showed a 15-cm multilobulated, heterogeneous, enhanced mass in the RLQ, which we suspected originated from the colon. He underwent a right hemicolectomy, and the pathologic result was a colon mesenteric UPS. We report a rare case of a primary UPS of the colon mesentery and discuss the characteristics of this neoplasm in reference to the literature.

Undifferentiated pleomorphic sarcoma of the orbital region: a case report / Sarcoma pleomórfico indiferenciado da região orbitária: relato de caso

ABSTRACT Undifferentiated pleomorphic sarcoma (UPS) is an extremely rare tumor that occurs in the head and neck region. Here, we report a unique case of a primary undifferentiated pleomorphic sarcoma in the orbital region. A 35-year-old woman presented with a progressive proptosis and periocular edema for 1 year. She had no previous history of surgery, skin malignancy, or radiation. Imaging tests showed an extraconal mass, not involving the muscles. The tumor was surgically removed and adjuvant radiotherapy was required after histological examination, which showed an undifferentiated pleomorphic sarcoma of the orbit. There was no recurrence after 1 year of follow-up. Though rare, undifferentiated pleomorphic sarcoma should be included in the differential diagnoses of orbital tumors.

RESUMO O sarcoma pleomórfico indiferenciado (SPI) é um tumor extremamente raro na região da cabeça e pescoço. Relatamos um caso de um sarcoma pleomórfico indiferenciado primário na região orbital. Uma mulher de 35 anos apresentou proptose progressiva e edema periocular há um ano. Ela não tinha histórico prévio de cirurgia, malignidade da pele ou radiação. Exames de imagem mostraram uma massa extraconal, poupando os músculos. O tumor foi removido cirurgicamente e foi necessária radioterapia adjuvante após o resultado histopatológico. O exame histológico demonstrou um sarcoma pleomórfico indiferenciado da órbita. Não houve recidiva após 1 ano de seguimento. Apesar de raro, o sarcoma pleomórfico indiferenciado deve ser incluído no diagnostico diferencial de qualquer tumor originado na órbita.

A Case of Myxofibrosarcoma in the Cheek / 대한이비인후과학회지

Myxofibrosarcoma is the most common soft tissue sarcoma that occurs in late adult life, mainly occurring in the lower extremities and trunk. However, head and neck myxofibrosarcoma is extremely rare. The most reliable treatment of adult soft tissue sarcoma is surgical resection with negative margin. A 79-year-old man presented with a left cheek mass first detected six months ago. The pathologic report of the mass showed that it was myxofibrosarcoma and consequently postoperative radiotherapy was done. However, distant and locoregional metastasis occurred postoperatively. We report this case with a brief review of literature.

Histiocitoma fibroso maligno metastásico en estómago / Metastatic malignant fibrous histiocytoma in the stomach

El histiocitoma fibroso maligno es el sarcoma de partes blandas más frecuente de la adultez tardía. Se desarrolla habitualmente en los miembros inferiores y en el retroperitoneo. Su pronóstico es malo, la recidiva local y las metástasis a distancia. Se presenta el caso de un paciente con metástasis en el estómago de un histiocitoma fibroso maligno. El objetivo de esta publicación es la presentación de una forma poco común de diseminación metastásica del histiocitoma fibroso maligno(AU)

Malignant fibrous histiocytoma is the most common soft tissue sarcoma of late adult life. It usually develops in the lower extremities and in the retroperitonium. Prognosis is fairly poor; local relapse and distant metastasis are the common natural history. The is presented of a patient with stomach metastasis of malignant fibrous histiocytoma. This paper is aimed at presenting a rare way of metastatic dissemination of the malignant fibrous histiocytoma(AU)

Malignant fibrous histiocytoma in a patient presenting with urinary system symptoms

Summary Malignant fibrous histiocytoma is a rare tumor. It is most commonly seen in individuals between the fifth and seventh decades of life, in extremities, and less frequently in the retroperitoneum. Although its etiology is not clearly known, radiotherapy, chemical agents, previous history of surgery, trauma and fracture, and Hodgkin lymphoma have been blamed. Leiomyosarcoma, liposarcoma and rhabdomyosarcoma should be taken into account in differential diagnosis. It is seen on computed tomography as a mass lesion with irregular borders and density similar to that of the surrounding muscle tissue. Necrotic and hemorrhagic components in the mass are characterized as heterogeneous low density areas. Fluid-fluid levels can be detected by computed tomography and magnetic resonance imaging.

Histiocitoma fibroso maligno cerebral en paciente pediátrico: Presentación de un caso y revisión de la literatura / Malignant Fibrous Histiocytoma of the Brain in Pediatric Patient: A Case Report and Literature Review

Los tumores cerebrales son la segunda causa de neoplasias malignas en niños y los sarcomas primarios en el sistema nervioso central son extremadamente raros. Por esta razón, presentamos los resultados del estudio de un caso en una menor de edad y la revisión realizada sobre la fisiopatología, manifestaciones clínicas y hallazgos en las imágenes de resonancia magnética cerebral de esta patología. Se trata de una niña de 7 años de edad que ingresa al servicio de urgencias con cefalea, sintomatología sugerente de hipertensión endocraneana y disminución de la fuerza del hemicuerpo derecho, por lo que se le practica resonancia magnética cerebral (RM) con medio de contraste, con hallazgo de masa extraaxial temporoparietal izquierda, que desplaza la línea media. La paciente es valorada por neurocirugía y remitida a craneotomía frontoparietal izquierda, con resección completa del tumor. El informe de patología muestra sarcoma fusocelular de alto grado, compatible con histiocitoma fibroso maligno.

Brain tumours are the second cause of malignant neoplasms in children while primary sarcomas in the central nervous system (SNC), are extremely rare. For this reason, we present the results of the study of a case of a girl and revision on the pathophysiology, clinical manifestations, and findings on brain MRI of this pathology. We report the case of a sevenyear- old patient admitted to the emergency department with headache and symptoms suggestive of intracranial hypertension, and decreased strength of the right side of the body. In brain MRI, we found a left temporoparietal extra-axial mass, displacing the midline. The patient was examined by the neurosurgeon who performed a left frontoparietal craniotomy, with complete tumor resection. The pathology study showed high-grade spindle cell sarcoma, classified as malignant fibrous histiocytoma.