Tratamiento multidisciplinario de un sarcoma recurrente de la fosa isquiorrectal / A successful multidisciplinary approach to a recurrent ischiorectal fossa sarcoma

Los tumores de la fosa isquiorrectal son poco frecuentes, habitualmente descritos en reportes o series de casos. Estas lesiones son un reto terapéutico, requiriendo un estudio preoperatorio apropiado, además de discusión y manejo guiado por un comité multidisciplinario, que permiten lograr resultados óptimos tanto oncológicos como funcionales. Presentamos un caso de un hombre de 73 años con antecedentes de resección de un tumor glúteo izquierdo en otro centro 5 años antes. La biopsia fue compatible con un sarcoma epiteloideo (SE) de alto grado, con margen quirúrgico < 0,5 mm que requirió ampliación de los márgenes posteriormente. Además, se realizó radioterapia adyuvante. Al cuarto año de seguimiento el paciente desarrolla dolor e induración con retracción en relación a cicatriz quirúrgica, siendo objetivada una recidiva tumoral local. Luego de una discusión multidisciplinaria, se realizó una desfuncionalización con ileostomía en asa laparoscópica y resección tumoral con preservación del ano y del piso pélvico. El defecto fue cubierto por el equipo de cirugía plástica utilizando un colgajo perforante de la arteria glútea superior. La biopsia confirmó la recidiva tumoral y los márgenes quirúrgicos fueron negativos. El paciente es dado de alta a los 25 días postoperatorios por cuidados del colgajo, sin complicaciones. Al año de seguimiento el paciente no presenta recidiva tumoral, la ileostomía fue cerrada, y sus resultados funcionales en términos defecatorios y de la herida son buenos.

Ischiorectal fossa tumors are rare lesions, mostly described in case reports or case series. These lesions represent a diagnostic and therapeutic challenge. Hence, an appropriate preoperative study and multidisciplinary discussion are essential to achieve good oncologic and functional results. We report a case of a 73-year-old male operated on five years before in another health center due to the diagnosis of a left gluteal tumor. The lesion was excised, and biopsies confirmed a high-grade epithelioid sarcoma with a close margin, requiring a subsequent wider excision of the surgical margins. The patient received adjuvant radiotherapy. After four years of follow-up, the patient developed mild pain with skin retraction around the former incision. A local recurrence was diagnosed by imaging. In a multidisciplinary team meeting, a decision to resect the lesion with preservation of the anus and the pelvic floor was taken. The patient underwent a laparoscopic defunctioning loop ileostomy and a resection of the recurrent tumor in the ischiorectal fossa with preservation of the anal sphincter. The defect was covered utilizing a superior gluteal artery perforator flap and a partial gluteus maximus muscle rotation. The tumor was completely excised with negative margins. The patient was discharged without complications after 25 days due to flap management. After one year of follow-up, the patient is recurrence-free, and the ileostomy was closed.

Lipoma gigante: tratamento cirúrgico / Giant lipoma: surgical treatment

Introdução: Lipomas gigantes (>1000g), embora raros, têm importância médica relevante, pois podem acometer qualquer região do corpo e ser diagnosticados em fases avançadas, nas quais há comprometimento funcional. Eles também podem ser confundidos com neoplasias malignas de tecidos moles. Ademais, a história natural de surgimento e evolução de lipomas gigantes ainda não é exatamente conhecida, o que justifica a realização de estudos adicionais. O objetivo do corrente estudo é apresentar os resultados do tratamento cirúrgico de um caso de lipoma gigante. Relato de Caso: Relato de caso ocorrido no serviço de cirurgia plástica do Hospital Roberto Santos em Salvador, Bahia, no ano de 2016. Homem de 62 anos, lavrador, 1,80m de altura, 74kg, com tumoração dorsal de 35cm de diâmetro, avaliado por meio de história clínica e exame de tomografia computadorizada e submetido a excisão elíptica e fechamento em plástica em W. Não houve complicações pós-operatórias. A histopatologia confirmou o diagnóstico de lipoma gigante (2881g). Os resultados pós-cirúrgicos foram considerados excelentes pelo paciente e equipe cirúrgica. Conclusão: O lipoma gigante foi removido satisfatoriamente sem complicações, com excelentes resultados funcionais e estéticos.

Introduction: Giant lipomas (>1000g), although rare, have relevant medical importance, as they can affect any region of the body and are diagnosed in advanced stages, in which there is functional impairment. They can also be confused with malignant soft tissue neoplasms. Furthermore, the natural history of the emergence and evolution of giant lipomas is still not exactly known, which justifies further studies. The current study aims to present the results of the surgical treatment of a case of giant lipoma. Case Report: Case report that occurred in the plastic surgery service of the Roberto Santos Hospital in Salvador, Bahia, in the year 2016. A 62-year-old male farmer, 1.80 m tall, 74 kg, with a dorsal tumor measuring 35 cm in diameter, evaluated through clinical history and computed tomography examination and submitted to elliptical excision and plastic closure in W. There were no postoperative complications. Histopathology confirmed the diagnosis of giant lipoma (2881g). Post-surgical results were considered excellent by the patient and surgical team. Conclusion: The giant lipoma was satisfactorily removed without complications, with excellent functional and aesthetic results.

Leiomioma primario de páncreas en una gestante: presentación de caso / Primary pancreatic leimyoma in a pregnant woman: case report

RESUMEN Introducción: Los tumores pancreáticos en el embarazo son muy raros, el diagnóstico y tratamiento en este grupo de pacientes es complejo. Objetivo: Presentar el primer caso de un leiomioma primario del páncreas en una gestante. Caso clínico: Embarazada de 26 años de edad, sin antecedentes relevantes. Primigesta, que se encontraba cursando la semana 13 de gestación. Durante una evaluación ecográfica de rutina, se le detecta una tumoración en la glándula pancreática. Se remitió a la consulta multidisciplinaria de atención a la materna y en conjunto deciden realizar la interrupción voluntaria del embarazo. Luego la paciente fue remitida a la consulta de la especialidadde Cirugía General. Al recibir a la misma, se quejaba de dolor en epigastrio e hipocondrio izquierdo, además de hipo ocasional. Al examen físico se encontró una tumoración palpable en epigastrio. Se realizaron exámenes analíticos básicos sin encontrarse alteraciones, en los estudios de imagen realizados, ecografía abdominal y tomografía contrastada de abdomen se demostró una tumoración en el cuerpo y cola del páncreas. Se planificó tratamiento quirúrgico electivo, se realizó pancreatectomía distal con esplenectomía, por invasión del estómago, se complementó con gastrectomía subtotal. La paciente evoluciona sin complicaciones. La biopsia informó un tumor mesenquimal benigno primario de páncreas, tipo leiomioma, bien encapsulado. Conclusiones: Se presenta el primer caso de un leiomioma primario del páncreas en una gestante. Los tumores pancreáticos en embarazadas son enfemedades raras, el manejo por un equipo multidisciplinario es la mejor forma de obtener resultados satisfactorios para la paciente.

ABSTRACT Introduction: Pancreatic tumors during pregnancy are very rare, diagnosis and treatment are always difficult. Objective: To present the first case of a primary pancreatic leiomyoma in a pregnant woman. Case report: A 26 years old primiparous pregnant is reported, with no relevant backgrounds. She was in her first trimesters of pregnancy. Diagnosed with a pancreatic tumor during a routine evaluation. She was referred to a multidisciplinary consult, deciding the voluntary interruption of pregnancy, then she is evaluated by the General Surgery specialty clinic for complaining of epigastric pain and hiccup. On physical examination an epigastric tumor was found. Auxiliary exams were normal. Imagenologic studies confirm a tumor in the pancreatic body and tail. Surgical treatment was scheduled, distal pancreatectomy with splenectomy, due to invasion of gastric wall subtotal distal gastrectomy was performed. Patient evolution without complications. Biopsy reported a well capsulated, leiomyoma type, primary mesenchymal pancreatic tumor. Conclusions: The first case of a primary pancreatic leiomyoma in a pregnant woman is presented. Pancreatic tumors during pregnancy are rare. Multidisciplinary approach is the best way to improve results.

Fibrous hamartoma of infancy with sarcomatous transformation: an unusual morphology

ABSTRACT Background Fibrous hamartoma of infancy (FHI) is a rare soft tissue lesion arising as a subcutaneous mass involving the axilla, trunk, and upper arm in infants and children <2yrs. Sarcomatous transformation in FHI is described in anecdotal cases in the literature. Case Report We describe one such example arising as a mass in the lower back in a 3-month-old infant. On histology, the tumor contained classic triphasic morphology; however, brisk mitotic activity noted at multiple foci was diagnostically challenging to categorize. The tumor was evaluated for ETV6-NTRK3 fusion to exclude other common differentials. Conclusion While FHI may be frequently encountered in infants, rare sarcomatous transformation are known to occur and merits special attention as it can be misdiagnosed. Also, a close follow-up is warranted as the lesion is known to recur locally.

Choice of bone reconstruction methods and materials after chondrosarcoma resection / 中国组织工程研究

BACKGROUND:The prognosis of chondrosarcoma is closely associated with tumor location, size, removal methods and biological characteristics. OBJECTIVE:To explore the choice of reconstruction materials for bone defects after chondrosarcoma resection based on the retrospective analysis of different resection methods. METHODS:We retrospectively analyzed clinical data from 82 cases of chondrosarcoma who had received hondrosarcoma resection at the Department of Orthopedics, Changzhen Hospital of Second Military Medical University from January 1999 to January 2010. Al the involved patients were confirmed pathological y as having chondrosarcoma, including 51 males and 31 females, with a mean age of 39.8 years (range, 15-68 years). Chondrosarcoma was found in the pelvis (n=26), spine (n=19), femur (n=19), tibia and fibula (n=8), and shoulder (including the humerus) (n=12). Preoperative X-ray, MRI or CT was taken as wel as ECT. Clinical data, resection methods, and fol ow-up results were col ected for multivariate analysis to screen the possible prognostic factors of chondrosarcoma. RESULTS AND CONCLUSION:For chondrosarcoma, different types of resection result in a quite difference in recurrence rates, metastasis and mortality rates. The initial resection which is done outside the boundaries can significantly reduce the recurrence rate, metastasis rate and mortality. Autogenous bone, al ograft bone, and prosthetic reconstruction materials are effective for functional reconstruction. Autologous bone graft is limited clinical y because of its limited sources and complications at donor site. Al ograft or prosthetic reconstruction of bone defects is an effective method. Limited number of clinical cases reported makes it difficult to determine the pros and cons of different reconstruction materials. The need for reconstruction depends on the defect site and size, and reconstruction method depends on the type of resection. The important criteria for the choice of reconstruction materials include both form and functional repair of bone defects, chondrosarcoma type and the patient’s request.

Clinical prospect of research on molecular targeted therapy in soft tissue sarcomas / 国际肿瘤学杂志

It has been suggested that some unusual molecules and genes might be involved in the development of soft tissue sarcomas (STS) and that drugs targeting specific molecules and genes would selectively kill tumor cells, which have been demonstrated to be more effective approach with less side effect than traditional chemotherapy. However, most of the effective targets of STS are currently not elucidated, and the effects of trial-target therapy are mostly not satisfactory, thus multiple targeting drugs might guide a future direction for tumor therapy.

Dermatofibrossarcoma protuberans gigante de couro cabeludo: relato de caso / Giant dermatofibrosarcoma protuberans of scalp: case report

O dermatofibrossarcoma protuberans (DFSP) é uma neoplasia de tecidos moles de notávelagressividade local, altos índices de recorrência (> 40%) e baixo potencial metastatizante.Ocorre principalmente em tronco e porção proximal de extremidades, sendo incomum emcouro cabeludo, onde perfaz menos de 5% do total de casos. Habitualmente apresenta dimensõesmédias de 5 cm à ocasião da ressecção cirúrgica. O objetivo deste trabalho é relatarcaso de paciente com tumoração gigante em couro cabeludo (20x16x14 cm), enfatizandosuas peculiaridades e discutindo possibilidades terapêuticas.

Dermatofibrosarcoma protuberans (DFSP) is a soft tissue neoplasm with remarkable localaggressiveness, high levels of recurrence (> 40%) and low metastasizing potential. Occurspredominantly in trunk and proximal extremities, being uncommon in scalp, where representsless than 5% of all cases. Usually presents average size of 5 cm at the time of surgicalexcision. The objective of this study is to report a patient with giant tumor of scalp (20x16x14cm), emphasizing its peculiarities and therapeutic options.