Fibromatosis tipo desmoide de apéndice cecal: reporte de caso inusual y revisión de la literatura / Appendicular desmoid tumor: unusual case and review of the literature

RESUMEN Antecedente : La fibromatosis tipo desmoide es un proceso neoplásico benigno no encapsulado localmente invasivo y agresivo, que se origina de la proliferación de fibroblastos y miofibroblastos aparentemente normales. La localización más frecuente de la fibromatosis es extra-abdominal (60%), pared abdominal (25%) e intra-abdominal (8-15%), en raras ocasiones puede originarse en las vísceras (0,73%), como el páncreas, unión gastroesofágica, diafragma y apéndice. La incidencia anual de tumor desmoide se estima de 2 a 5 casos por millón. En el presente artículo, reportamos un caso de presentación inusual, originado en el apéndice cecal. Caso : Paciente de sexo femenino de 41 años con dolor pélvico agudo, que ingresa a sala de operaciones con diagnóstico clínico y ecográfico de probable tumor de ovario a pedículo torcido. En el intraoperatorio se evidenció una tumoración sólida de 15 cm de diámetro que dependía del apéndice cecal, correspondiendo el estudio anatomopatológico a tumor desmoide de apéndice cecal. Conclusiones : El tumor desmoide puede originarse en diversas localizaciones extra e intra-abdominales, siendo esta última la más rara y agresiva. El diagnóstico preoperatorio exacto es muy difícil y casi siempre los pacientes ingresan al quirófano con sospecha diagnostica de tumoración intraabdominal de etiología desconocida. Los factores de riesgos asociados a su aparición aún no se encuentran caracterizados, siendo la resección quirúrgica completa del tumor -con márgenes quirúrgicos libres (R0)- el tratamiento de elección; sin embargo, el riesgo de recurrencia es alto incluso con la remoción óptima del tumor.

ABSTRACT Background : Desmoid type fibromatosis is a benign neoplastic process non-encapsulated locally invasive and aggressive, which arises from a proliferation of bland-looking fibroblasts and myofibroblasts. The most frequent location of fibromatosis is extra- abdominal (60%), abdominal wall (25%) and intra-abdominal (8-15%), rarely can originate in the viscera (0.73%), such as the pancreas, gastroesophageal junction, diaphragm and appendix. The annual incidence of desmoid tumor is estimated at 2 to 5 cases per million. In this article, we report a case of unusual presentation, originated in the cecal appendix. Case : A 41-year-old female patient with acute pelvic pain, admitted to surgical ward with a clinical and ultrasound diagnosis of probably ovarian pedicle tumor. An explorative laparotomy revealed a solid mass of 15 cm in diameter arising from the cecal appendix, with the anatomopathological study corresponding to a desmoid tumor of the cecal appendix. Conclusions : The desmoid tumor can arise from several extra and intra-abdominal locations, the latter being the rarest and most aggressive. Accurate preoperative diagnosis is very difficult and almost always patients enter to surgery with suspected diagnosis of intraabdominal tumor of unknown etiology. The risk factors associated to its appearance are not yet characterized. The complete surgical resection of the tumor -with free surgical margins (R0)- is the treatment of choice; however, the risk of recurrence is high even with optimal tumor removal.

CT and MRI findings of desmoid-type fibromatoses / 实用放射学杂志

Objective To investigate the CT and MRI features of desmoid-type fibromatoses(DF).Methods The images of 29 ca-ses with DF proved by pathology were reviewed retrospectively.CT and MRI examinations were performed in 1 5 cases respectively, and both in 1 case.Results The median age was 33.0 years with a male-to-female sex ratio of 1 ︰ 2.2.36 lesions were discovered,among which 26 cases were solitary while 3 cases had multiple focus.1 9 lesions were extra-abdominal,14 ones in the abdominal wall and 3 ones intra-abdominal.The largest diameter of tumors was 13 -1 75 mm.27 lesions displayed ovoid or elongated shape while 9 ones were irregular .The boundary could be clear or unclear.25 lesions extended along muscle planes with consistent long axis.23 focuses grew aggressively,1 6 of which crossed the muscle clearance,while 13 lesions grew expansively.On CT,20 lesions appeared iso-den-sity or slightly hypo-density compared with normal muscles,mostly homogeneous(1 5/20).Most lesions exhibited gadually moderate or obvious enhancement on post-contrast CT,and the enhancement was homogeneous or heterogeneous.1 7 lesions were found on MRI,which presented signal intensity similar to or slightly higher than that of muscles on T1 WI and most were homogeneous(12/1 7);on T2 WI,all were heterogenous with slightly high or high intensity.All lesions demonstrated obvious enhancement on MRI after contrast administration ,and most were heterogeneous(12/1 7).Sheets or bands of low signal were detected within 1 1 lesions with no enhancement.Conclusion DF has some characteristics on CT and MRI,which are valuable for accurate pre-operative diagnosis,and for evaluation of the lesion extent and involvement of adjacent structures.

Fibromatosis agresiva de la pared torácica asociada a implante mamario: a propósito de un caso / Aggressive fibromatosis of the chest wall associated to mammary implant: a case report

La fibromatosis agresiva es una lesión benigna, que comprende el 0,3% de todos los tumores sólidos. A pesar que la pared torácica es un lugar común, este tipo de tumor raramente se ha asociado con los implantes o tejido mamarios. Pocos casos se han descrito en conjunción con un implante de seno. Se presenta el caso de una paciente femenina de 27 años, quien le aparece esta patología dos años después de la colocación de implante mamario

The aggressive fibromatoses is a benign lesion, is not frequent, represent the 0.3% of the all solid tumors. The chest wall was considered a common place for tumors, but this type is rarely associated with the mammary implants. There are few cases reported in conjunction with a mammary implant. We presented a rare clinical case of a feminine patient of 27 years old, to whom apparition of this pathology two years after the collocation of mammary implant

Paquidermodactilia: Presentación de un caso / Pachydermodactyly: case report

La paquidermodactilia (pachy: grueso; dermos: piel: dactylos: dedos) es un tipo de fibromatosis digital benigna, patología rara que afecta sobre todo a varones jóvenes. Su etiología no se conoce bien, pero algunos autores defienden los traumatismos repetidos como principal desencadenante. Presentamos el caso de un varón de 16 años con engrosamiento digital difuso en ambas manos de 6 años de evolución, asintomático. El paciente refirió tener un "tic" en las manos; consiste en el roce muy frecuente entre los dedos. Se trata de una patología benigna cuyo diagnóstico es clínico, por lo que no es necesario realizar pruebas adicionales que prolonguen el número de visitas del paciente al hospital y los trastornos que ello conlleva. Se debe hacer hincapié en el control del hábito por parte del paciente, para evitar traumatismos repetidos y lograr la mejoría del cuadro.

Pachydermodactyly is a type of benign digital fibromatoses, a rare condition that affects mostly young men. The cause is unknown but some authors suggest that traumatism can be involved. A 16-year-old man presented with symmetrical painless swellings on the fingers of both hands. He used to rub his fingers together. The control of living habits must be enphasized in order to avoid repeteated injuries. Hence, our consultation work is with the patient and his family. We describe a new case of pathology with a common clinical diagnosis, whose ignorance could lead us to carry out unnecesary tests involving inconvenience to the patient. It is important to emphasize the modification of the patient's living habits in order to achieve its improvement.

A rare case of superficial polyfibromatosis in a Filipino male adolescent

The superficial fibramatoses are a rare group of mainly benign disorders that clinically manifest as slow progressive dermal hypertrophies. This bewildering group of dermal matrix proliferation is composed of fibrous tissue, fibroblastic cells or spindle stromal cells with varying degrees of cellularity. Skin lesions are usually solitary or localized to a specific site of predilection. This is a case of a 15-year-old Filipino boy, presenting with asymptomatic firm, skin colored to erythematous papules and nodules on his limbs and nose. The lesions slowly worsened, resulting in flexion deformity and limited range of motion of both hands.