Melanosis peritonei in pregnancy: a case report and review of literature
© Springer-Verlag Berlin Heidelberg 2013
Received: 11 February 2013
Accepted: 30 October 2013
Published: 13 November 2013
KeywordsCysts Laparotomy Melanosis peritonei Peritoneal melanosis Pregnancy
Peritoneal melanosis is a rare condition characterized by pigment deposition in the peritoneum. Benign peritoneal melanosis may occur in association with other congenital cystic conditions such as ovarian dermoid, peritoneal, enteric duplication cysts, or even gastric triplication. We report the case of melanosis peritonei found during pregnancy diagnosed at the time of cesarean section for fetal distress.
Melanosis peritonei is an extremely rare condition with only 13 previously reported cases in the English literature [1–3]. This is the first published case in pregnancy. The condition has to be differentiated from other pigmented lesions like endometriosis, hemosiderosis, melanoma, and lipofuscinosis affecting the peritoneum.
The condition, when not associated with any malignancy, almost exclusively affects young females (mean age 18.3 ± 10.3 years; range: 0.5–28 years) with only 1 of the 14 reported cases was male. It has also been observed in the elderly, but this was always in association with malignancy [2, 3].
Commonly involved organs include ovaries, peritoneum, omentum, appendix, enteric cyst, intestine, liver, and gall bladder. The condition is associated with ovarian lesions in 57 % of the cases . In the remainder, it was associated with gastrointestinal congenital conditions and very rarely, with metastatic melanoma (one case) and colonic malignancy (one case) [2, 3]. The availability of a frozen section biopsy of the lesion may expedite the decision to proceed with a definitive surgical treatment in cases of benign lesions, which in our case, was not possible.
The pathogenesis of peritoneal melanosis, in particular, the origin of the pigment-producing cells is unclear. Among the recognized theories, spillage of pigment from ruptured ovarian teratomas is the most accepted . The majority has indeed been observed in women with ovarian melanogenic tumors or ovarian teratomas in the reproductive age group. However, this theory is unable to explain the association of peritoneal melanosis with other conditions like peritoneal cyst, duplication cyst of the gastrointestinal tract, or colonic carcinoma. A possibility of multifocal occurrence has been suggested as melanin deposits have been noted on both inner and outer surfaces of the viscera. It has been suggested that peritoneal melanosis might occur independent of ovarian melanogenic tumors . Further, the presence of melanocytes in the peritoneal cavity may be attributed to aberrant development from the neural crest cells as a result of either excessive migration of the neural crest cells toward the anterior abdominal wall or due to anomalous malposition of neural crest to the peritoneum before the 10th week [6, 7]. Others have postulated that the esophageal mucosa, a common site for melanosis within enteric cysts, and a likely source of melanin, ruptures to peritoneal spaces . The pigment could be derived from the breakdown of heme within dermoid cysts possessing a fully developed gastric wall with peptic ulceration . These theories do not explain, however, the absence of melanophages in the mucosa of the enteric cyst, or unruptured cystic lesions. Lastly, it has been suggested that the pinched-off mesothelial remnants during development might be the progenitor cells evoking peritoneal melanosis .
On diagnosis, pathology will determine further management. Malignancy is to be excluded and suggested treatment for the dermoids is oopherectomy . Metastatic peritoneal malignant melanoma has a very poor prognosis and can be treated with chemotherapy, with few survivors beyond 2 years of diagnosis .
Melanosis peritonei is an extremely rare condition that may be incidentally encountered during the course of a laparotomy. We did so at the time of cesarean section. It is usually associated with ovarian lesions. If not, it may point to other congenital anomalies and very rarely, to metastatic melanoma or colonic malignancy.
Conflicts of interest
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