Sister Mary Joseph's Nodule as a First Sign of Cancer of the Cecum: Report of a Case

Abstract

Key words: Sister Mary Joseph; Umbilical metastasis; Cecum cancer]C utaneous metastases of malignant neoplasms are not common, occurring between 0.7 and 9 percent of autopsy evaluations. 1 Those to the umbilicus, also known as Sister Mary Joseph's nodule, are even more rare and represent only 10 percent of all secondaries to the skin. 2 These nodules usually arise from the gastrointestinal or genitourinary tract and may represent the first sign of a previously unknown primary tumor. This case demonstrates a Sister Mary Joseph's nodule caused by an unknown primary cancer of the cecum. REPORT OF A CASEA 66-year-old female presented with a six-month history of umbilical lump, initially with a diameter of 0.5 cm, covered by erythematous skin. This was initially treated with conservative measures by a dermatologist, who three months later performed a biopsy. Histology showed cutaneous metastasis caused by a primary occult adenocarcinoma.Five months after the onset of the umbilical lesion, although the diagnostic work-up was still in progress, the patient required an emergency admission for intestinal obstruction. Physical examination of the abdomen showed an erythematous umbilical nodule (diameter of 1.5 cm) with irregular and ill-defined edges, attached to the underlying layers, and of fibrous consistency (Fig. 1). At laparotomy, there was a cecal neoplastic lesion attached to the anterior peritoneum in the umbilical area. A right colectomy with complete excision of the umbilical lesion was performed. Histology of the operative specimen showed a moderately differentiated adenocarcinoma. The skin and subcutaneous tissue specimen presented a central raised lesion consisting of metastases of the primary adenocarcinoma. Pathologic staging was pT3 pN2 pM1. After discharge, the patient received six courses of doxorubicin and died 21 months after surgery. DISCUSSIONUmbilical neoplastic nodules are rare and can be caused by 1) primary tumor (38 percent of cases), 2) endometriosis (32 percent), and 3) metastases (30 percent).

Sister Mary Joseph’s nodule that originated from lung adenocarcinoma

“…There have been various reports of survival of patients with SMJN, but most of them revealed that SMJN patients often had a poor probability of survival, which was approximately 10-21 months. 6,8,11 Although the pathogenesis of SMJN is not well elucidated, possible pathways of metastasis to the umbilicus are as follows: (1) direct invasion through the peritoneum, the ligamentum teres hepatis, or the falciform ligament; (2) lymphatic spread; (3) hematogenous metastasis. 5,6,8,11 Among these pathways, direct invasion is the most common route of metastasis because of the defect in the muscular layer and subcutaneous fat at the umbilicus.…”

“…The primary lesion for SMJN is usually an intraabdominal organ, including the gastrointestinal tract, pancreas, gallbladder, and female genital organs, [6][7][8][9] although a few cases of SMJN with the primary tumor in the breast or esophagus have been reported. 10,11 Regarding lung cancer, Heatley and Toner reviewed 33 cases of SMJN between 1970 and 1984, with only two cases of metastatic lung adenocarcinoma being noted.…”

Metastatic neuroendocrine malignancy presenting as Sister Mary Joseph’s nodule

“…Overall metastases to the umbilicus represent only 10% of all secondary tumours, which have spread to the skin [5,6]. In 15% to 30% of patients, the source of the primary site of the tumour is unknown [7][8][9] Presentation is usually as a painful or painless lump in the umbilicus. The size of the nodule usually ranges from 0.5 to 2 cm, although some nodules may reach up to 10 cm in size [10].…”

Sister Mary Joseph Nodule

“…21,26,35 Recent studies have demonstrated that there is a better survival (21 months) for patients if they are treated with a combination of surgery and adjuvant therapy instead of surgery alone (7.4 months) or chemotherapy alone (10.3 months). 18,23,27,43 Despite increasing patient survival by 11 to 13 months, some authors still propose only palliative treatment because of the poor long-term prognosis. 12,20,44 Early diagnosis is imperative for successful cancer treatment.…”

“…Ishrat et al 38 reported that after a positive histologic diagnosis of metastatic carcinoma (from SMJN), the mean survival time was 10 months, 38 and many other authors have reported similar results. 12,21,23,[39][40][41][42][43] Management of these cases is controversial, with some clinicians advocating aggressive surgery and adjuvant therapy. 21,26,35 Recent studies have demonstrated that there is a better survival (21 months) for patients if they are treated with a combination of surgery and adjuvant therapy instead of surgery alone (7.4 months) or chemotherapy alone (10.3 months).…”