Sister Mary Joseph's nodule as presenting sign of a desmoplastic small round cell tumor

Abstract

Umbilical metastases, also named Sister Mary Joseph's nodules, are well documented in the adult population and most often represent an underlying intra-abdominal malignancy, usually a carcinoma of gastrointestinal or gynecologic origin. They are indicative of widespread abdominal disease and are associated with a poor prognosis. An extensive review of the literature reveals only two such presentations in the pediatric population. A 14-year-old male presented with an umbilical mass, which was found to be a metastatic lesion of a desmoplastic small round cell tumor (DSRCT) of the abdomen. The diagnosis of an intra-abdominal malignancy, most commonly a DSRCT, should be considered in the presence of an umbilical mass in a child.

Sister Mary Joseph nodule caused by metastatic desmoplastic small round cell tumor: A clinicopathological report

“…The occurrence of a Sister Mary Joseph nodule metastasizing from the DSRCT is very rare. Table I compares the present rare case of DSRCT with three reported cases of Sister Mary Joseph nodule caused by metastatic DSRCT that have been previously described in the literature (5)(6)(7). The presence of Sister Mary Joseph nodule often means a poor prognosis, with a median survival time of 6 months for metastatic DSRCT.…”

“…DSRCT is frequently found in the abdominal cavity, although cases with involvement of the thoracic cavity, paratesticular area, kidney, head and neck region have been reported (1,(8)(9)(10). The most frequently presenting symptoms of abdominal DSRCT are vague abdominal pain, gradual enlargement of the abdominal mass, abdominal distension, abdominal pain, weight loss and other symptoms associated with obstruction of the intestinal or urinary tract (5)(6)(7)(8)(9)(10). The most common site of metastasis is the regional abdominal lymph node.…”

“…Improved survival rates are associated with complete resection of the tumor. Poor survival rates, with a median survival time of 6 months, are associated with Sister Mary Joseph nodule caused by metastatic DSRCT (5)(6)(7). Until more effective forms of treatment are found, the authors of this case study recommend multimodality treatment, including chemotherapy, surgery and radiotherapy.…”

Desmoplastic Small Round Cell Tumour (DSRCT) - A Case Report and Review of Literature

“…2 Desmoplastic small round cell tumor is an aggressive cancer. 2,10,11,12 It is less common and less reported in pediatric age group. Desmoplastic small round cell tumor is classified as a soft tissue sarcoma.…”