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

Abstract

Sister Mary Joseph nodule is an uncommon metastatic intra-abdominal malignancy involving the umbilicus. The present study describes a rare case of desmoplastic small round cell tumor (DSRCT), histological grade 3, high grade, Gilly classification 4, stage IV, in an 18-year-old Thai man presenting with the Sister Mary Joseph nodule, ascites and pleural effusion. The histopathological examination of the umbilical mass revealed the presence of malignant small round cells associated with prominent stromal desmoplasia. Immunohistochemical stains showed positive reactivity to cytokeratin, desmin, neuron-specific enolase, Wilms' tumor 1, CD56, CD99 and SWI/SNF-related matrix-associated actin-dependent regulator of chromatin subfamily B member 1 (SMARCB1)/INI1 in the small round cells. Fine needle aspirations of the ascitic fluid and pleural effusion were performed, and immunocytochemistry revealed a metastatic DSRCT. The patient received a VDC/IE regimen of chemotherapy, comprising vincristine, doxorubicin, and cyclophosphamide alternating with ifosfamide and etoposide; however, the patient developed systemic metastasis and succumbed to the disease 6 months later.

Unusual Presentation of Wilms' Tumor as Umbilical Granuloma

“…[3] Intra-abdominal malignancy is known to cause umbilical deposits in adults, also called Sister Mary Joseph's nodule, secondary to spread through the lymphatics and venous channels draining along the abdominal wall. [4] This phenomenon is not known in children, and the umbilical mass, in this case, was soft and granulomatous as opposed to hard and skin covered. It would appear that the two conditions existed coincidentally but the persistence of the granuloma at 7 months age led to This is an open access journal, and articles are distributed under the terms of the Creative Commons Attribution-Non Commercial-ShareAlike 4.0 License, which allows others to remix, tweak, and build upon the work non-commercially, as long as appropriate credit is given and the new creations are licensed under the identical terms.…”

Benign Umbilical Tumors Resembling Sister Mary Joseph Nodule

“…Oozing or ulcerative changes on the surface indicate that a malignant tumor is undergoing necrotic degeneration due to its rapid growth. 6 Similarly, multiple, widely distributed satellite lesions reflect malignancy. 24 …”

“…The “benign umbilical tumor” group comprised 19 patients. The “Sister Mary Joseph nodule” group consisted of 30 patients: 2 from our department and 28 from a PubMed search using the keyword “Sister Mary Joseph nodule [title].” 4 - 25 …”

“…Oozing or ulcerative changes on the surface indicate that a malignant tumor is undergoing necrotic degeneration due to its rapid growth. 6 Similarly, multiple, widely distributed satellite lesions reflect malignancy. 24 In this study, none of the benign umbilical tumors were fixed, whereas most of the Sister Mary Joseph nodules (93.3%; 28/30) showed a firm and fixed consistency upon physical examination, indicating packed cellularity and invasion into the adjacent tissues, both of which are characteristics of malignancy.…”