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mucosal small Bowel metastasis from uterine leiomyosarcoma

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					IMAJ • VOL 12 • MAy 2010                                                                                                              case cOmmunicatiOns




mucosal small Bowel metastasis from uterine
leiomyosarcoma
Offir Ben-Ishay MD1, Pavel Shmulevsky MD1, Eran Brauner MD1, Euvgeny Vladowsy MD2 and Yoram Kluger MD FACS1
1
  Department of General Surgery B and 2Institute of Pathology, Rambam Health Care Campus, affiliated with Rappaport Faculty of Medicine, Technion-Israel Institute of Technology,
Haifa, Israel




                                                                          motherapy is well established, but there is          tant metastases were evident at that time.
 KeY wOrds: uterine leiomyosarcoma, mucosal                               no consensus regarding the optimal man-              Histopathology examination showed
            small bowel metastases, abnormal                              agement of these patients. Metastases are            spindle cell leiomyosarcoma with areas
            [18F]-2-fluoro-deoxy-D-glucose uptake                         common and recurrence rates are high,                of myxoid and pleomorphic cells with
                                              IMAJ 2010; 12: 309–310      possibly due to the presence of microme-             a mitotic index of 10–15 mitotic figures
                                                                          tastases at the time of diagnosis.                   per 10 high power fields. At the same
                                                                              We present here a patient who was                time chest computed tomography scan
                                                                          diagnosed with leiomyosarcoma of the                 revealed a lesion suspected to be a metas-

                    u enchymal neoplasm composed
                           terine leiomyosarcoma is a mes-                uterus presenting with distant meta-                 tasis in the lower lobe of the left lung.
                                                                          static spread in a rare location.                    This was confirmed by PET-CT and a
                    of smooth muscle. It is the most com-                                                                      segmental lobar metastasectomy was
                    mon uterine pure mesenchymal tumor,                                                                        performed. Histopathology revealed a
                    although it represents only 5% of uterine             Patient descriPtiOn                                  metastatic lesion of the primary uterine
                    malignancies [1]. It affects 0.4/100,000              A 60 year old woman was admitted to                  leiomyosarcoma. A local recurrence at
                    women a year [2]. Owing to the aggres-                hospital in March 2009 for exploratory               the same site was found and resected
                    sive nature of this tumor, women with                 laparotomy due to abnormal uptake on                 at the end of the same year. In 2007, a
                    uterine sarcomas have a poor prognosis                positron emission tomography with                    right upper lobe metastasis was discov-
                    with an overall survival of 50% at 2 years,           radiolabeled [18F]-2-fluoro-deoxy-D-                 ered by routine follow-up PET-CT scan.
                    even when diagnosed early [1]. Surgical               glucose scan, revealing the presence of              Consequently, another segmental lobar
                    resection is the treatment of choice and              a small lesion suspected to be a meta-               metastasectomy was performed.
                    may be curative for lesions confined to the           static leiomyosarcoma in the left lower                  In 2008, a routine PET-CT showed
                    uterus. The role of radiotherapy and che-             abdominal quadrant.                                  increased FDG uptake in the left lobe of
                                                                              Her medical history was remarkable               the thyroid suspected to be a metastatic
                                                                          for recurrent metastatic uterine leiomyo-            lesion. The patient underwent a partial
[a] Abnormal focal FDG uptake in left lower quadrant presumed             sarcoma that was repeatedly treated                  thyroidectomy and again histopathol-
to be metastatic lesion by (transaxial) PET-CT
                                                                          with wide local excisions. In 1993 at the            ogy revealed a metastatic lesion of leio-
                                                                          age of 44 and still premenopausal, the               myosarcoma.
                                                                          patient was diagnosed with symptom-                      In March 2009 a follow-up PET-CT
                                                                          atic uterine myomas. Due to her morbid               scan showed increased FDG uptake in
                                                                          obesity and three prior cesarean sec-                the left lower abdominal quadrant sus-
                                                                          tions, follow-up only was prescribed.                pected to be a small metastatic lesion
                                                                              In 2005, at the age of 56, she was               [Figure A]. At laparotomy no peritoneal,
                                                                          admitted for evaluation of abdominal                 hepatic, retroperitoneal or intestinal sero-
                                                                          pain. Diagnostic workup revealed a large             sal spread was found. Thorough manual
                                                                          pelvic mass causing partial small bowel              examination of the small bowel revealed
                                                                          obstruction. On surgical exploration a               a small (3 cm) intraluminal polypoid
                                                                          large (24 cm) pelvic mass originating                mass at mid-jejunum. Segmental small
                                                                          from the uterus was revealed. A total                bowel resection was performed and the
                                                                          abdominal hysterectomy and bilateral
                                                                                                                                   PET-CT = positron emission tomography-
                                                                          salpingo-oopherectomy was performed.                     computed tomography
                                                                          Neither enlarged lymph nodes nor dis-                    FDG = [18F]-2-fluoro-deoxy-D-glucose


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case cOmmunicatiOns                                                                                                                 IMAJ • VOL 12 • MAy 2010




                                                             for 25% of uterine mesenchymal              patients who present with abnormal
[B] Leiomyosarcoma, infiltrating mucosa of small intestine
                                                             tumors, endometrial stromal sarcomas        FDG uptake in the abdominal cavity
hematoxylin and eosin, x50
                                                             for 15% and mixed mullerian tumors          and carefully searched for during explo-
                                                             for 50% of the tumors, the latter being     ration. Even though leiomyosarcoma is
                                                             referred to as metaplastic carcinoma        an aggressive and lethal tumor, it can
                                                             (carcinosarcoma) [4]. Sarcomas most         be treated with routine follow-up and
                                                             commonly invade and spread locally,         aggressive intervention for metastatic
                                                             but may have an aggressive growth           spread if and when it occurs.
                                                             pattern with lymphatic and hemato-
                                                             genous spread. Micrometastases are          correspondence:
                                                                                                         dr. O. Ben-ishay
                                                             often present at the time of diagnosis.
                                                                                                         Dept. of General Surgery B, Rambam Health Care
                                                             The most common sites for metastatic        Campus, Haifa 31096, Israel
                                                             spread are the peritoneal cavity and the    Phone: (972-4) 856-2231
                                                                                                         email: o_ben-ishay@rambam.health.gov.il
                                                             omentum (30–50%), lung (30–40%)
                                                             and liver (10%) [3]. Metastases to the      references
                                                             heart, pericardium, skin, stomach and       1. Denschlag D, Masoud I, Stanimir G, Gilbert L.
                                                             pancreas have also been described.             Prognostic factors and outcome in women with
                 patient recovered uneventfully. The his-        Metastases to the gastrointestinal         uterine sarcoma. Eur J Surg Oncol 2007; 33(1): 91-5.
                                                                                                         2. Brown L. Pathology of uterine malignancies. De-
                 topathology report confirmed that the       tract from extra-abdominal sites are un-       partment of Histopathology, Leicester Royal
                 mass was a metastatic lesion of spindle     common. Malignant melanoma and car-            Infirmary, Leicester, UK, Clinical Oncology (2008)
                 cell leiomyosarcoma with pleomorphic        cinoma of the breast and lung are the          20: 433e447 doi:10.1016/j.clon.2008.04.005
                                                                                                         3. Melone GA, D'Elia A, Brogna C, Salvati M.
                 areas of uterine origin [Figure B].         most common malignancies spreading             Uterine leiomyosarcoma metastatic to the brain:
                                                             to the gastrointestinal tract.                 case report. Tumori 2008; 94(6): 856-60.
                                                                 Small bowel mucosal metastases          4. Mawrin C, Kirches E, Dietzmann K, Weis S.
                 cOmment                                     from uterine leiomyosarcomas are ex-           Uterine leiomyosarcoma metastatic to the brain
                                                                                                            stem. Arch Gynecol Obstet 2002; 266(2): 119-21.
                 Uterine sarcomas are rare tumors that       tremely rare. To the best of our knowl-     5. Gerst PH, Levy J, Swaminathan K, Kshettry
                 account for 5% of all uterine malignan-     edge, only one prior case was reported in      V, Albu E. Metastatic leiomyosarcoma of the
                 cies. Mean age at presentation is 55        the English-language literature [5]. This      uterus: unusual presentation of a case with late
                                                                                                            endobronchial and small bowel metastases.
                 years [3]. Leiomyosarcomas account          rare occurrence should be suspected in         Gynecol Oncol 1993; 49(2): 271-5.