Unusual contents of a scrotal swelling

Morris‐Stiff, Gareth; Falk, Gavin A.; Joyce, Daniel; Chalikonda, Sricharan

doi:10.1136/bcr.03.2011.4023

Cited by 3 publications

(2 citation statements)

References 8 publications

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“…En la enfermedad menos avanzada, los síntomas locales se observan en 50-80% de los pacientes, sin ascitis gelatinosa y pudiera corresponder al sitio del tumor primario, como síntomas de apendicitis, como en el 25% de los casos, o la ubicación de los implantes peritoneales, incluyendo dolor abdominal bajo, presión pélvica y dolor ginecológico en mujeres debido a los depósitos de mucina del tumor ovárico mucinoso en el 20-30% de los pacientes [22][23] . Por úl-timo, el PMP se encontró casualmente en hasta el 20% de los pacientes sometidos a procedimientos tales como laparotomías, laparoscopía o ecografía para otras condiciones médicas, por ejemplo, hernia 24 , tumor de vejiga 25 , en orina 26 , prolapso utero-vaginal total 27 , cáncer de recto recurrente 28 , embarazo 29 y en cesárea 30 .…”

Section: Manifestaciones Clínicasunclassified

Untitled

2017

Rev Med UAS

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Resumen:El pseudomixoma peritoneal, consiste en la presencia de implantes generalizados de material mucinoso en cavidad abdominal como depósitos tumorales en todo el peritoneo, cuya etiología más frecuente es la rotura espontánea o intraoperatoria de una neoplasia mucinosa apendicular de bajo grado de malignidad. La enfermedad regularmente es diagnosticada después de los 40 años. Se presenta clínicamente con una variedad de signos y síntomas inespecíficos. La tomografía computada es el método diagnóstico más utilizado, ya que brinda un aumento en las densidades del líquido mucinoso, en comparación con el líquido no mucinoso y brinda una planeación preoperatoria con fines pronóstico. El tratamiento tradicional consiste en cirugía de citorreducción. La estrategia actual de tratamiento consiste en cirugía única de citorreducción en combinación con quimioterapia intraperitoneal hipertérmica.Palabras clave: Pseudomixoma peritoneal, perforación apendicular, clasificación Abstract:The peritoneal pseudomixoma consists in the presence of generalized implantation of mucinous material in the abdominal cavity as of tumoral deposits in all the peritoneum, which most frecuent etiology is the spontaneous or intraoperatory rupture of a low grade of malignancy mucinous apendicular neoplasm. The disease is regularly diagnosed after 40 years of age. Its clinical presentation is very inespecific. The computed tomography is the most used diagnostic method because it denotates an increase in the densities of the mucinous fluid in comparison with the non mucinous fluid and gives a preoperatory planeation with pronostic goals. The traditional treatment consists in cytoreduction. The current treatment strategy consists of surgery in combination with intraperitoneal hyperthemal chemotherapy. El pseudomixoma peritoneal (PMP), con una incidencia estimada de 1-2 casos en un millon 6 , consiste en la presencia de implantes generalizados de material mucinoso en la cavidad abdomi-

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Section: Manifestaciones Clínicasunclassified

Untitled

2017

Rev Med UAS

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“…Finally, PMP is coincidentally found in up to 20% of patients undergoing such procedures as laparotomy, laparoscopy or imaging for other medical conditions, e.g. hernia [ 17 - 27 ], bladder tumor [ 28 ], jelly like material in urine [ 29 ], total uterovaginal prolapse [ 30 ], recurrent rectal cancer [ 31 ], pregnancy [ 32 ] and cesarean section [ 33 ]. PMP cases presenting with an ulcerated skin fistula on the right flank [ 34 ] or a subcutaneous nontender umbilical nodule [ 35 ] have also been reported.…”

Section: Introductionmentioning

confidence: 99%

Secreted mucins in pseudomyxoma peritonei: pathophysiological significance and potential therapeutic prospects

Amini

Masoumi-Moghaddam

Ehteda

et al. 2014

Orphanet J Rare Dis

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Pseudomyxoma peritonei (PMP, ORPHA26790) is a clinical syndrome characterized by progressive dissemination of mucinous tumors and mucinous ascites in the abdomen and pelvis. PMP is a rare disease with an estimated incidence of 1–2 out of a million. Clinically, PMP usually presents with a variety of unspecific signs and symptoms, including abdominal pain and distention, ascites or even bowel obstruction. It is also diagnosed incidentally at surgical or non-surgical investigations of the abdominopelvic viscera. PMP is a neoplastic disease originating from a primary mucinous tumor of the appendix with a distinctive pattern of the peritoneal spread. Computed tomography and histopathology are the most reliable diagnostic modalities. The differential diagnosis of the disease includes secondary peritoneal carcinomatoses and some rare peritoneal conditions. Optimal elimination of mucin and the mucin-secreting tumor comprises the current standard of care for PMP offered in specialized centers as visceral resections and peritonectomy combined with intraperitoneal chemotherapy. This multidisciplinary approach has reportedly provided a median survival rate of 16.3 years, a median progression-free survival rate of 8.2 years and 10- and 15-year survival rates of 63% and 59%, respectively. Despite its indolent, bland nature as a neoplasm, PMP is a debilitating condition that severely impacts quality of life. It tends to be diagnosed at advanced stages and frequently recurs after treatment. Being ignored in research, however, PMP remains a challenging, enigmatic entity. Clinicopathological features of the PMP syndrome and its morbid complications closely correspond with the multifocal distribution of the secreted mucin collections and mucin-secreting implants. Novel strategies are thus required to facilitate macroscopic, as well as microscopic, elimination of mucin and its source as the key components of the disease. In this regard, MUC2, MUC5AC and MUC5B have been found as the secreted mucins of relevance in PMP. Development of mucin-targeted therapies could be a promising avenue for future research which is addressed in this article.

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