Although uterine leiomyosarcoma (ULMS) is a rare tumor of the female reproductive system (constituting only 1% of all uterine malignancies), it is a highly malignant and rapidly progressing neoplastic disease [1] [2] [3]. A specific risk factor for ULMS is yet to be identified, but various reports have associated obesity, diabetes mellitus, previous pelvic radiation, and the use of tamoxifen with an increased risk for this type of malignancy [4] [5]. The clinical presentation is somewhat nonspecific, but abnormal vaginal bleeding is the most important complaint, as it is observed in up to 90% of cases [1]. In addition to vaginal bleeding, other notable symptoms are pelvic pain or pressure in the area (predominantly because of uterine enlargement, which is seen in 70% of women) and dysmenorrhea [1] [4]. ULMS is exclusively seen in perimenopausal and menopausal women, and despite the fact that up to 60% of patients are diagnosed when the tumor is still confined to the uterus, the overall prognosis is quite poor [1] [4] [5]. The primary reason is a very high rate of tumor recurrence and a poor response to adjuvant radiation and/or chemotherapy, but a delayed diagnosis is equally responsible for such outcomes [2] [6]. The 5-year survival rates range from 76% in early stages to 29% when distant metastases are present, implying that an early diagnosis may drastically improve the outlook [5].

• Thyroid Nodule

  We report the case of a 64-year-old woman presenting a fast growing thyroid nodule whose primitive or metastatic origin was not obvious, for which 18F-FDG PET/CT helped in the diagnostic process and in the later management of the patient. [ncbi.nlm.nih.gov]

• Subcutaneous Nodule

  At debulking surgery, a pelvic mass measuring 10 cm was observed, and a subcutaneous nodule measuring 4 cm was noted at the site of the right accessory port, with intact underlying peritoneum. [ncbi.nlm.nih.gov]

• Cerebellar Ataxia

  The Karnofsky performance scale (KPS) was 40 with left hemiparesis and cerebellar ataxia. She was treated by resection of the left occipital and cerebellar tumors, followed by γ-knife irradiation of the residual tumors. [ncbi.nlm.nih.gov]

• Ataxia

  The Karnofsky performance scale (KPS) was 40 with left hemiparesis and cerebellar ataxia. She was treated by resection of the left occipital and cerebellar tumors, followed by γ-knife irradiation of the residual tumors. [ncbi.nlm.nih.gov]

• Vaginal Bleeding

  The clinical presentation is somewhat nonspecific, but abnormal vaginal bleeding is the most important complaint, as it is observed in up to 90% of cases. [symptoma.com]

  The clinical presentations include vaginal bleeding, pelvic pain or pressure, and awareness of an abdominal-pelvic mass. The diagnosis should be considered if rapid uterine enlargement occurs, especially in a post-menopausal woman. [ncbi.nlm.nih.gov]

• Enlarged Uterus

  The transvaginal ultrasonography showed an enlarged uterus with an 8 8 cm uterine solid mass, partially vasculated. [karger.com]

  On May 15, computed tomography ( ct ) imaging of the abdomen and pelvis showed an enlarged uterus, with multiple fibroids. The patient was then booked for elective surgery. [current-oncology.com]

The diagnosis of a uterine leiomyosarcoma must be made early on, and perimenopausal or menopausal women who report abnormal vaginal bleeding must be thoroughly investigated to exclude ULMS as a possible cause. Firstly, a detailed patient history needs to be obtained, which will focus on the timing and progression of symptoms, whereas a complete physical examination, with an emphasis on abdominal and pelvic palpation, is the second step of the workup. If initial signs and symptoms point to a pathology of the female reproductive tract, imaging procedures must be carried out. A heterogeneous uterine mass is detected on standard abdominal ultrasonography (US), which may be a useful initial method, but the diagnosis of a ULMS requires more advanced diagnostic studies [6]. Computed tomography (CT), but more frequently magnetic resonance imaging (MRI), are mandatory in determining the stage of the tumor [1] [5]. According to the International Federation of Gynecology and Obstetrics (FIGO) classification system, ULMS is categorized into four stages [5]:

• Stage I - The tumor is confined to the uterus, with a diameter of ≤ 5 cm (IA) or >5 cm (IB).
• Stage II - Extension of the tumor outside of the uterus, either in the adnexa (IIA) or other structures within the pelvis (IIB).
• Stage III - Tumor invades one (IIIA) or more than one structure in the abdomen, or metastatic dissemination into the pelvic and/or paraaortic lymphatic systems.
• Stage IV - Presence of metastatic deposits in the rectum and/or bladder (IVA) or distant sites, such as the lungs (IVB) are hallmarks of this stage.

Biopsy and subsequent histopathological examination with immunohistochemistry should be performed whenever possible in order to confirm the exact type of the tumor. Cytologic atypia, extensive mitotic activity and the presence of necrosis are typical for ULMS, whereas the expression of androgen, estrogen and progesterone receptors, c-KIT, p53 and Ki67 is seen on immunohistochemistry [2] [7].

1. El-Khalfaoui K, du Bois A, Heitz F, Kurzeder C, Sehouli J, Harter P. Current and future options in the management and treatment of uterine sarcoma. Ther Adv Med Oncol. 2014;6(1):21-28.
2. Menczer J, Schreiber L, Berger E, Ben-Shem E, Golan A, Levy T. CA125 expression in the tissue of uterine leiomyosarcoma. Isr Med Assoc J. 2014;16(11):697-679.
3. Ducie JA, Leitao MM. The role of adjuvant therapy in uterine leiomyosarcoma. Expert Rev Anticancer Ther. 2016;16(1):45-55.
4. Vellanki VS, Rao M, Sunkavalli CB, Chinamotu RN, Kaja S. A rare case of uterine leiomyosarcoma: a case report. J Med Case Reports. 2010;4:222.
5. Hensley ML, Barrette BA, Baumann K, et al. GCIG Consensus Review: Uterine and Ovarian Leiomyosarcomas. Int J Gynecol Cancer. 2014;24(9 Suppl 3):S61-S66.
6. Nagai T, Takai Y, Akahori T, et al. Novel uterine sarcoma preoperative diagnosis score predicts the need for surgery in patients presenting with a uterine mass. Springerplus. 2014;3:678.
7. Aster, JC, Abbas, AK, Robbins, SL, Kumar, V. Robbins basic pathology. Ninth edition. Philadelphia, PA: Elsevier Saunders; 2013.