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An umbilical nodule in a patient with enlarged uterus

      1. Indication

      A previously healthy 59-year-old female presented with weakness in the lower extremities and abdominal distension of one month. She also experienced unintentional weight loss of 20 pounds within 3 months. The physical examination was remarkable for the presence of two well-circumscribed reddish nodules over the umbilicus (Fig. 1, panel A), marked abdominal distension, and motor power of 1/5 in both legs. Computed tomography (CT) findings are shown in Fig. 1, panel B&C.
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      Fig. 1Sister Mary Joseph's nodule with distended abdomen in our patient. The nodule was firm, not tender, and measured 2.5 × 3 cm (Panel A). Coronal computed tomography of the chest, abdomen, and pelvis with contrast showed a markedly distended uterus containing heterogeneous intermediate attenuation material with subtle septation and areas of nodular thickening (Panel B). Axial computed tomography revealed a contrast-enhancing subcutaneous nodule at the umbilicus, compatible with Sister Mary Joseph's nodule (Panel C).
      What is the diagnosis?

      2. Diagnosis

      A computed tomography of the chest, abdomen, and pelvis revealed an enlarged uterus, measuring 31.2 cm in length, with multiple metastases involving the thoracolumbar vertebral bodies, peritoneum, pelvic lymph nodes, and liver. The patient received intravenous dexamethasone and emergency surgical decompression of the spinal cord. Surgical bone specimens showed poorly differentiated malignant cells with necrosis. A hysteroscopic biopsy of the endometrial tissue revealed carcinosarcoma of the uterus on histopathologic assessment.
      Sister Mary Joseph's nodule is a rare cutaneous metastasis from an advanced internal malignancy into the umbilicus. It is seen in 1–3% of abdomino-pelvic malignancies [
      • Wronski M.
      • Klucinski A.
      • Krasnodebski I.W.
      Sister Mary Joseph nodule: a tip of an iceberg.
      ]. The proposed mechanisms include direct extension of the tumor to the umbilicus as well as lymphatic or hematogenous spread. This unique finding, via a meticulous physical examination, can help us diagnose the underlying cancer. The condition is usually associated with peritoneal spread and poor prognosis.
      With an incidence of only 5% of uterine malignancies, uterine carcinosarcoma is rare, but it is an aggressive neoplasm [
      • Arend R.
      • Doneza J.A.
      • Wright J.D.
      Uterine carcinosarcoma.
      ]. Surgical resection is the standard management. A combined chemo-radiotherapy can act as an adjuvant treatment [
      • Gurumurthy M.
      • et al.
      An update on the management of uterine carcinosarcoma.
      ]. However, due to distant metastases and extensive local invasion, the tumor was unresectable. The patient decided to pursue hospice care without any further aggressive measures.

      Contributors

      SW, MM and SP were involved in the evaluation and treatment of the patient. SW and SP wrote the manuscript. All authors approved the final manuscript.

      All source of support

      None.

      Conflict of interests

      The authors declare no competing financial interests.

      References

        • Wronski M.
        • Klucinski A.
        • Krasnodebski I.W.
        Sister Mary Joseph nodule: a tip of an iceberg.
        J Ultrasound Med. 2014; 33: 531-534
        • Arend R.
        • Doneza J.A.
        • Wright J.D.
        Uterine carcinosarcoma.
        Curr Opin Oncol. 2011; 23: 531-536
        • Gurumurthy M.
        • et al.
        An update on the management of uterine carcinosarcoma.
        Obstet Gynecol Surv. 2011; 66: 710-716