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      Radiation Therapy for Sister Mary Joseph's Nodule: A Review

      review-article
      , MD, PhD a , b , * , , MD, PhD a , , MD, PhD c , , MD, PhD c
      Advances in Radiation Oncology
      Elsevier

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          Abstract

          Purpose

          Umbilical metastasis, known as Sister Mary Joseph's nodule (SMJN), is a manifestation of advanced malignant disease. Patients with SMJN usually require supportive care or palliative systemic chemotherapy. However, with the increasing number of older and infirm patients, radiation therapy for SMJN is needed more frequently. Therefore, we conducted this review to provide insights into radiation treatment for this rare condition.

          Methods and Materials

          We searched PubMed on October 16, 2022, and obtained 275 articles that described SMJN or metastatic tumors within or near the umbilicus, as well as 255 case reports or case series (298 patients) and 20 reviews, original articles, or other study types, 1 of which also described a case.

          Results

          The prognosis of patients with SMJN is extremely poor. However, some patients can survive for more than 2 years. The primary organs of the umbilical metastasis are mainly in the gastrointestinal tract, including the stomach, colon, and pancreas. In addition to these organs, the ovaries, uterine corpus, and breasts are the major organs affected in women. Metastasis may be divided into 4 types according to the tumor location and mechanism of the extension: within the umbilicus, not within although existing near or adjacent to the umbilicus, in the umbilical or paraumbilical hernia sac, and iatrogenic disease. Only 7 reports described patients who received radiation therapy in detail. The patients were divided into 2 groups: a relatively long course and high total dose (approximately 45 Gy) group, and a short course and low total dose group.

          Conclusions

          Umbilical metastasis, known as SMJN, is a rare disease and is divided into 4 types based on the location of the disease and extent mechanism. Although the prognosis of the disease is poor, some patients survive for more than 2 years. Only 7 case reports precisely described radiation therapy. Half of the patients were treated with a short course, whereas the other half were treated with relatively high doses of up to 45 Gy.

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          Most cited references45

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          Palliative radiation therapy for bone metastases: Update of an ASTRO Evidence-Based Guideline.

          The purpose is to provide an update the Bone Metastases Guideline published in 2011 based on evidence complemented by expert opinion. The update will discuss new high-quality literature for the 8 key questions from the original guideline and implications for practice.
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            Metastases to soft tissue: a review of 118 cases over a 30-year period.

            Metastatic tumors presenting as soft tissue masses are relatively rare and can be the source of diagnostic confusion both clinically and pathologically. The authors' experience was reviewed at a large academic medical center over a 30-year period (1971-2000) with metastases to soft tissue. The tumors in the study included mainly lesions involving skeletal muscle or skeletal muscle and subcutaneous tissue of the upper and lower limbs, trunk, shoulders, and buttocks. Direct extension from tumors originating in bone or adjacent organs, tumors involving the skin or areas known to contain abundant lymph nodes (ie, axilla, groin), and hematopoietic malignancies were excluded. One hundred and eighteen cases were identified; 60 patients were women and 58 were men. The age range was 20 to 87 years (median of 53.5 years). The primary tumor was located in the skin (19 patients), lung (13 patients), breast (13 patients), kidney (12 patients), colon and rectum (12 patients), uterus (8 patients), ovary (5 patients), head and neck (tongue, pharynx, larynx, nasal cavity, and mandible) (5 patients), esophagus (2 patients), stomach (2 patients), cervix (2 patients), small bowel (2 patients), bone (2 patients), adrenal gland (1 patient), eye (1 patient), testis (1 patient), urinary bladder (1 patient), and salivary gland (1 patient). In 27% (32 of 118 cases) of cases, the soft tissue metastasis was the initial manifestation of the disease. In 13.5% (16 of 118 cases) of cases the primary site of origin could not be identified. The sites of metastasis included the abdominal wall (25 patients), back, including scapular region (20 patients), thigh (17 patients), chest wall (15 patients), arm (15 patients), shoulder (11 patients), buttock (5 patients), perineum (3 patients), leg (2 patients), foot (1 patient), umbilical area (1 patient), ankle (1 patient), scalp (1 patient), and elbow (1 patient). The histologic classification of the tumors included carcinoma (83 patients), malignant melanoma (20 patients), sarcoma and carcinosarcoma (9 patients), malignant mixed Mullerian tumor (2 patients), seminoma (1 patient), malignant teratoma (1 patient), malignant gastrointestinal stromal tumor (1 patient), and neuroblastoma (1 patient). Many of the tumors displayed histologic features that created difficulties for diagnosis and could be easily mistaken on routine histopathologic examination for a variety of primary soft-tissue sarcomas. Routine use of immunohistochemical stains aided in their proper recognition. Metastases are not an infrequent finding in soft tissue and they may represent the initial manifestation of the disease. Use of a basic panel of immunohistochemical stains is recommended for defining the cell type and arriving at the correct diagnosis. 2007 American Cancer Society
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              Umbilical metastasis or Sister Mary Joseph's nodule.

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                Author and article information

                Contributors
                Journal
                Adv Radiat Oncol
                Adv Radiat Oncol
                Advances in Radiation Oncology
                Elsevier
                2452-1094
                23 July 2023
                February 2024
                23 July 2023
                : 9
                : 2
                : 101321
                Affiliations
                [a ]Department of Radiation Therapy, Misugikai Sato Hospital, Hirakata, Osaka, Japan
                [b ]Department of Radiation Oncology, Juntendo University, Graduate School of Medicine, Bunkyo, Tokyo, Japan
                [c ]Department of Surgery, Misugikai Sato Hospital, Hirakata, Osaka, Japan
                Author notes
                [* ]Corresponding author: Keisuke Sasai, MD, PhD ksasai@ 123456juntendo.ac.jp
                Article
                S2452-1094(23)00149-5 101321
                10.1016/j.adro.2023.101321
                10885576
                38405321
                57a95f68-fa23-414c-8b49-765809839758
                © 2023 The Authors

                This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

                History
                : 4 April 2023
                : 13 July 2023
                Categories
                Critical Review

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