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Uterine Leiomyosarcoma

Uterus Leiomyosarcoma

Uterine leiomyosarcomas are tumors arising from the myometrium and are primarily encountered in perimenopausal and postmenopausal women. Abnormal vaginal bleeding is often the only symptom. The diagnosis, unfortunately, is often made in the advanced stages of the disease and so a poor overall prognosis is observed. Imaging studies and biopsy are necessary steps during the workup.


Presentation

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].

Aspiration
  • The breast mass was palpable and a fine needle aspiration (FNA) was performed.[ncbi.nlm.nih.gov]
  • Meconium aspiration syndrome This is a case of meconium aspiration syndrome. Radiopaedia Danke![pacs.de]
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]
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]
  • Although the most frequent symptoms are vaginal bleeding and abdominal pain, the symptoms are generally associated with dimensions and localization of the tumor.[ncbi.nlm.nih.gov]
  • 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]
  • In this report, we describe the case of 49-year-old woman with a history of "uterine fibroids," who presented with vaginal bleeding and a positive urine pregnancy test.[ncbi.nlm.nih.gov]
  • Patients with uterine leiomyosarcoma (LMS) typically present with vaginal bleeding, pain, and a pelvic mass, with atypical presentations of hypercalcemia and eosinophilia also being reported.[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 presence of symptoms such as an enlarged uterus, vaginal bleeding, abdominal distension, urinary frequency, etc., in a postmenopausal woman with a prior history of leiomyomas should raise the suspicion of leiomyosarcoma.[karger.com]

Workup

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].

Treatment

  • The rarity of LMS has led to a lack of consensus regarding appropriate treatment.[ncbi.nlm.nih.gov]
  • Patients with high pre-treatment CRP serum levels showed impaired OS compared to women with low levels (5-year-OS rates: 22.6% and 52.3%, p 0.007).[ncbi.nlm.nih.gov]
  • In the recent years, angiogenetic inhibitors have emerged for the treatment of several malignancies.[ncbi.nlm.nih.gov]
  • We identified a treatment-naive patient who has metastatic uterine leiomyosarcoma and has experienced complete tumor remission for 2 years on anti-PD-1 (pembrolizumab) monotherapy.[ncbi.nlm.nih.gov]
  • The principal role of chemotherapy is prevalently in the treatment of metastatic disease.[ncbi.nlm.nih.gov]

Prognosis

  • However, high-grade ESS with metastasis with YWHAE rearrangement shows a relatively favorable prognosis.[ncbi.nlm.nih.gov]
  • Although ULMS was associated with a high rate of complete or optimal surgery, the long-term prognosis was poor. Effective postoperative therapy should be developed to improve the long-term prognosis of patients with ULMS.[ncbi.nlm.nih.gov]
  • IMP3 expression in ULMS could be a marker of a poor prognosis.[ncbi.nlm.nih.gov]
  • The prognosis of uterine leiomyosarcoma (LMS) is notoriously poor and a standard chemotherapy for patients with uterine LMS has not yet been established.[ncbi.nlm.nih.gov]
  • Gamma-glutamyltransferase (GGT) is an established marker for proliferative/apoptotic balance and has been associated with cancer risk and prognosis.[ncbi.nlm.nih.gov]

Etiology

  • Significant blood eosinophilia is uncommon in leiomyomas and should raise suspicion of malignant etiology for a pelvic mass.[ncbi.nlm.nih.gov]
  • Because of their rarity, unknown etiology, and highly divergent genetic aberration, there is a lack of consensus on risk factors for occurrence and predictive poor outcomes as well as optimal therapeutic choices.[ncbi.nlm.nih.gov]
  • The case highlights the importance of considering infectious etiologies and Stauffer syndrome in the differential diagnosis of liver dysfunction in patients with intraabdominal malignancies.[ncbi.nlm.nih.gov]
  • The molecular etiology of uterine leiomyosarcoma (ULMS) is poorly understood, which accounts for the wide disparity in outcomes among women with this disease.[ncbi.nlm.nih.gov]
  • Patients with peripheral eosinophilia of unknown etiology should be screened for malignancy. Two cases of eosinophilia associated with uterine leiomyoma have been reported that disappeared following hysterectomy ( 15 ).[academic.oup.com]

Epidemiology

  • We used the SEER (Surveillance, Epidemiology, and End Results)-Medicare database to gather information on uterine LMS patients older than the age of 66 years diagnosed between 1992 and 2009.[ncbi.nlm.nih.gov]
  • Surveillance, epidemiology, and end results analysis of 2677 cases of uterine sarcoma 1989-1999. Gynecol Oncol. 2005;93:204-8. Dinh TA, Oliva EA, Fuller AF Jr., Lee H, Goodman A. The treatment of uterine leiomyosarcoma.[rarediseases.org]
  • Epidemiology (L.A.), Portuguese Oncology Institute-Porto, Portugal, Rua Dr. António Bernardino de Almeida Cancer Biology and Epigenetics Group (A.S.P.[journals.lww.com]
  • […] rarity of these tumors, large-scale epidemiologic studies have not been possible.[journals.lww.com]
Sex distribution
Age distribution

Pathophysiology

  • Abstract Fibrinogen has an important pathophysiological role in tumor cell progression and development of metastases in different types of cancer.[nature.com]

Prevention

  • The goal of this clinical research study is learn if taking Femara (letrozole) after a hysterectomy (surgical removal of the uterus) for uterine leiomyosarcoma will delay or prevent the cancer from coming back. Letrozole is an aromatase inhibitor.[clinicaltrials.gov]
  • Urine output was maintained at greater than 150 ml/15 minutes to prevent cisplatin-induced kidney damage. The heated chemotherapy solution was manually distributed in an attempt to maintain as uniform temperature as possible.[atlasofscience.org]
  • The goal is to prevent relapse or recurrence of their uterine leiomyosarcoma.[clinicaltrials.gov]
  • Surgery is a viable option in patients with disease limited to the uterus, but metastasis to the heart may require surgery to prevent acute and catastrophic complications.[karger.com]

References

Article

  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.

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Last updated: 2018-06-22 00:39