Uterine Myxoid Leiomyosarcoma Research Articles

Uterine Myxoid Leiomyosarcoma with Stromal Chondroid Metaplasia: A Rare Case Report

Background: Uterine leiomyosarcoma is a rare gynecological disease. Myxoid leiomyosarcoma (mLMS) is an aggressive and very uncommon type of leiomyosarcoma, with few cases reported in English literature. Stromal metaplasia is rare in leiomyosarcoma. Here we present huge uterine myxoid leiomyosarcoma with stromal chondroid metaplasia. Case Report: A 48–year–old single woman with lower abdominal pain and increased abdominal circumference. The detected mass on imaging was diagnosed as uterine mLMS with stromal chondroid metaplasia in the histopathological examination after surgery. Conclusion: Myxoid leiomyosarcoma should be considered in uterine mass with extensive myxoid change, infiltrative border, low mitotic count, and mild atypia. Stromal chondroid metaplasia can be seen in the myxoid leiomyosarcoma. [GMJ.2021;10:e1817]

Uterine Myxoid Leiomyosarcoma with Stromal Chondroid Metaplasia: A Rare Case Report

Background: Uterine leiomyosarcoma is a rare gynecological disease. Myxoid leiomyosarcoma (mLMS) is an aggressive and very uncommon type of leiomyosarcoma, with few cases reported in English literature. Stromal metaplasia is rare in leiomyosarcoma. Here we present huge uterine myxoid leiomyosarcoma with stromal chondroid metaplasia. Case Report: A 48–year–old single woman with lower abdominal pain and increased abdominal circumference. The detected mass on imaging was diagnosed as uterine mLMS with stromal chondroid metaplasia in the histopathological examination after surgery. Conclusion: Myxoid leiomyosarcoma should be considered in uterine mass with extensive myxoid change, infiltrative border, low mitotic count, and mild atypia. Stromal chondroid metaplasia can be seen in the myxoid leiomyosarcoma. [GMJ.2021;10:e1817]

Novel PLAG1 Gene Rearrangement Distinguishes a Subset of Uterine Myxoid Leiomyosarcoma From Other Uterine Myxoid Mesenchymal Tumors.

Genetic alterations in uterine myxoid leiomyosarcoma are unknown. We investigate the clinicopathologic features of 19 uterine tumors previously diagnosed as myxoid leiomyosarcomas in which tumoral RNA was subjected to targeted RNA sequencing. PLAG1, BCOR, BCORL1, HMGA2, and ALK break-apart fluorescence in situ hybridization (FISH) and BCOR, PLAG1, and ALK immunohistochemistry were performed in cases which failed or lacked fusions by sequencing. The diagnosis of myxoid leiomyosarcoma was confirmed in 15 cases after exclusion of 4 tumors with BCOR and ALK rearrangements. These 15 patients presented at a median age of 50 years with stage I (3), II (2), III (2), and IV (1) tumors, respectively; stage was unknown in 7 cases. Tumor size ranged from 10 to 24 cm. Matrix was myxoid in all tumors and also eosinophilic in 2. Cells were spindled, epithelioid, and both in 10, 2, and 3 tumors and showed mild, moderate, and severe nuclear atypia in 3, 8, and 4 tumors, respectively. Mitotic index ranged from <1 to 14/10 HPF, while tumor necrosis was present in 6 (40%). Novel TRPS1-PLAG1 or RAD51B-PLAG1 fusions were detected by sequencing in 4 tumors, 3 of which were also confirmed by FISH. Diffuse PLAG1 expression was seen in 7 tumors, including 4 with PLAG1 rearrangement. No morphologic differences were seen among PLAG1 fusion-positive and fusion-negative tumors. No PLAG1, HMGA2, ALK, BCOR, or BCORL1 rearrangements were detected by FISH in 11 tumors. On the basis of sequencing and FISH results, PLAG1 rearrangements resulting in PLAG1 expression underpin ~25% of myxoid leiomyosarcomas and may serve as a useful diagnostic biomarker. Immunohistochemistry, targeted RNA sequencing, and/or FISH may distinguish myxoid leiomyosarcoma from its morphologic mimics.

Abnormal p53 and p16 staining patterns distinguish uterine leiomyosarcoma from inflammatory myofibroblastic tumour.

Uterine myxoid leiomyosarcoma may show relatively bland histological appearances, despite its aggressive behaviour. Distinguishing uterine leiomyosarcoma from the more indolent inflammatory myofibroblastic tumour (IMT), which is amenable to targeted therapies, can be challenging. A significant subset of leiomyosarcomas harbour TP53 and/or CDKN2A genomic alterations. Here, we examined the diagnostic value of p53 and p16 immunohistochemistry in the distinction of uterine conventional and myxoid leiomyosarcoma from IMT, in correlation with targeted sequencing of TP53 and CDKN2A. We performed p53 and p16 immunohistochemistry in 49 tumours, including 23 uterine leiomyosarcomas (12 myxoid, 11 conventional) and 26 IMT (12 uterine, 14 extrauterine). TP53 and CDKN2A coding regions were sequenced in 20 cases (four myxoid, 11 conventional uterine leiomyosarcomas; four uterine, one extrauterine IMT). Abnormal p53 staining patterns (strong/diffuse or null) were observed in six of 12 (50%) myxoid and six of 11 (55%) conventional leiomyosarcomas but none of the IMT (P < 0.0001), correlating with TP53 mutation/deletion (P = 0.0001). P16 loss was detected in five of 10 (50%) myxoid and two of 11 (18%) conventional leiomyosarcomas, but none of the IMT (P = 0.0005), correlating with CDKN2A deletion (P = 0.014). Strong/diffuse p16 staining in six of 21 (29%) leiomyosarcomas and three of 26 (12%) IMT did not correlate with CDKN2A alterations. Abnormal p53 staining and p16 loss are observed frequently in uterine leiomyosarcomas, with 100% specificity and 70% sensitivity against IMT, and correlating with genomic alterations. Conversely, IMT shows normal p53 and p16 staining, highlighting the use of these markers in the differential diagnosis of uterine mesenchymal neoplasms.

Myxoid leiomyosarcoma of the uterus: a clinicopathological and immunohistochemical study of 10 cases

Uterine myxoid leiomyosarcoma (MLMS) is a rare tumor that requires modified diagnostic criteria compared with conventional leiomyosarcoma. We analyzed the clinicopathological and immunohistochemical features of 10 MLMS cases from a single institution. Nine of 10 MLMSs showed an invasive border, and 2 of 10 had vascular invasion. Seven cases exhibited low-grade nuclear features, low mitotic counts (median, 2/10 high-power fields), and no tumor cell necrosis. They were all at International Federation of Gynecology and Obstetrics stage I. Two recurred at the vaginal vault at 38 and 61 months, respectively. In contrast, 3 MLMSs with high-grade nuclei had a high mitotic rate (median, 12/10 high-power fields) and tumor cell necrosis (2/3). They were at an advanced International Federation of Gynecology and Obstetrics stage (IIIA-IVB). One had lung metastases at 6 months, and another died at 34 months. HMGA2 immunostaining was diffusely expressed in all MLMSs. Overexpression of p16 and IMP3 was present in 5 and 3 cases, respectively. We conclude that an invasive tumor border and p16 and/or IMP3 overexpression are helpful features in the diagnosis of MLMS. HMGA2 is a highly sensitive and useful marker for MLMS. MLMS might have 2 possible subtypes: high-grade, clinically aggressive MLMS and low-grade, relatively indolent tumors.

Uterine Myxoid Leiomyosarcoma with Paraneoplastic Syndrome: A Rare Combination

Myxoid leiomyosarcoma of uterus is a very rare malignancy with only 36 cases documented worldwide and no cases reported in the Indian subcontinent. The present case is of uterine myxoid leiomyosarcoma with associated pancytopenia that has not been described previously in the south-east asia and only one such case reported in world literature. A 32 year old woman who is a known case of uterine fibromyoma discovered in her previous pregnancy , presented to the opd with heavy bleeding per vagina. On examination, she had a large abdominal mass. Her hemoglobin was 6.3 g/dl and platelet count was 80,000/cu.mm and also developed leucopenia(4500/cu.mm).She had received five units of blood transfusion, but her haemoglobin and platelet counts remained low. Her bone marrow biopsy report came out to be inconclusive. She underwent laparotomy followed by total hysterectomy with bilateral salphingoophorectomy, omentectomy and appendicectomy. Histopathology reports showed uterine myxoid leiomyosarcoma ( FIGO stage IIIA). Her haematological profile improved considerably following the surgery and recurred with the recurrence of the disease. Hence, this is considered to be a case of pancytopenia due to paraneoplastic syndrome as a result of the myxoid leiomyosarcoma of the uterus.

Uterine myxoid leiomyosarcoma with tumor embolism extending into the right atrium.

Uterine myxoid leiomyosarcoma (MLMS) is an extremely rare variant of uterine leiomyosarcoma; only 56 cases were reported from 1982 to 2013. Uterine MLMS is characterized by a myxoid appearance and highly malignant behavior. We herein report a case involving a 65-year-old woman with uterine MLMS with a large tumor embolism that reached the right atrium. A total abdominal hysterectomy, bilateral salpingooophorectomy, and tumor embolism resection with the use of a heart-lung machine were performed. Epirubicin-ifosfamide chemotherapy in the adjuvant setting led to reductions in both the tumor emboli and peritoneal dissemination. The patient retained a good quality of life for 10 months after the initial surgery. She then developed progressive disease despite treatment with pazopanib. She died of her disease 14 months after the initial surgery. Although complete surgical resection of the tumor is desirable, tumor reduction surgery followed by adjuvant chemotherapy might help to retain a good quality of life. This is the first reported case of a primary uterine MLMS with tumor emboli.

Uterine Myxoid Leiomyosarcoma Associated with Multiple Myomas in a Fertile Woman: A Case Report

A 37 year old woman was admitted to our university hospital for menometrorrhagia. The case history showed that the patient had menometrorrhagia for six months; moreover, during the abdominal examination we found a mass occupying the hipogastric and mesogastric area. The tumefaction was hard and it reached the level of the umbilicus. On combined vaginal-abdominal examination a mass on the anterior wall and multiple myomata were felt; the uterus was found to have been enlarged to the size equivalent to 18 weeks pregnancy; adnexa regular were felt. During the surgery multiple myomas were found. The largest, 10 cm diameter, was soft in consistence with a gelatinous structure. Total abdominal hysterectomy with preservation of adnexa was performed (Figure 1). Histopathological result gave evidence of myxoid leiomyosarcoma in the largest myoma, whereas the others fibroid nodes were without atypia.

Co-existence of three rare gynecological tumors in a 79-year-old woman

The occurrence of two primary tumors simultaneously is relatively common. However, the occurrence of three kinds of gynecological tumors simultaneously is extremely rare. Here we presented a 79-year-old woman who had simultaneously three primary gynecological tumors which were relatively uncommon. Those tumors were myxoid leiomyosarcoma and endometrial mucinous adenocarcinoma in the uterus and a Leydig cell tumor in the right ovary. Uterine myxoid leiomyosarcoma is a very rare and aggressive variant of uterine sarcoma despite of its minimal even no atypia and a little mitotic activity. Mucinous endometrial adenocarcinoma is also a rare subtype which comprise only 0.6-5% of uterine cancers. Leydig cell tumor is a very rare ovarian tumor which is composed entirely or predominantly of Leydig cell.

Uterine Myxoid Leiomyosarcoma with Widespread Metastases in a Cat

A 10-year-old domestic shorthair cat was spayed to remove bilateral ovarian masses and an enlarged uterus. Both ovaries were effaced by large, irregular, firm, glistening, white cystic masses filled with clear viscous fluid. The uterine lumen was filled with copious amounts of clear viscous fluid, and the uterus contained multiple, firm, glistening, white nodules. Histologic examination revealed an invasive spindle cell neoplasm with features of malignancy, and extensive hypocellular areas containing an alcian blue-positive myxoid matrix. Tumor cells expressed caldesmon, alpha-smooth muscle actin, and estrogen receptor but were negative for desmin. The animal was euthanized 1 month later because of suspected local tumor recurrence. At necropsy, the abdominal cavity contained 120 ml of mucoid ascites; multiple tumor nodules were present in the abdominal and thoracic cavities. The clinical behavior and gross, microscopic, and immunohistochemical findings established a diagnosis of myxoid leiomyosarcoma.

Uterine Myxoid Leiomyosarcoma

In Brief Background: Myxoid leiomyosarcoma is a rare variant of uterine leiomyosarcoma, exhibiting malignant behavior despite the absence of cytologic atypia and of significant mitotic activity. Case: A 36-year-old woman presented with a 2-month history abdominal distention and discomfort. On pelvic examination, the uterus was noted to be 8-week size and a large mass approximately 14 × 9.8 × 9.8 cm was located posteriorly. Exploratory laparotomy was performed. Frozen section showed uterine myoma with degeneration. Permanent section, however, showed myxoid leiomyosarcoma of the uterus. Total abdominal hysterectomy with bilateral salpingooophorectomy was then performed. She was recovering well on her follow-up visit. Histopathologic report confirmed it to be uterine myxoid leiomyosarcoma. Conclusion: Myxoid leiomyosarcoma is an extremely rare variant of uterine sarcoma with a poor prognosis exhibiting malignant biologic behavior. Surgical resection of the tumor is the treatment of choice for such disease. Myxoid leiomyosarcoma is a rare variant of uterine leiomyosarcoma.

Massive myxoid leiomyosarcoma of the uterus

Uterine myxoid leiomyosarcoma is a very rare and aggressive variant of uterine sarcoma, of which only 26 cases have been described in the literature published in English. A 59-year-old woman, who had been menopausal for 5 years and had a huge abdominal mass underwent laparotomy; histopathology of the bulky tumor revealed the particular characteristics of a myxoid leiomyosarcoma. Immunohistochemically, Ki-67 contributed toward indicating a malignancy, whereas p53 and progesterone and estrogen receptors were negative. The level of serum Ca125 was high at diagnosis, within normal limits after the fifth cycle of chemotherapy, and subsequently increased again at recurrence. Sixteen months after surgery, the patient died of respiratory failure. Immunohistochemistry and Ca125 levels can aid the diagnosis, management, and prognosis of uterine myxoid leiomyosarcoma.

Massive myxoid leiomyosarcoma of the uterus

Uterine myxoid leiomyosarcoma is a very rare and aggressive variant of uterine sarcoma, of which only 26 cases have been described in the literature published in English. A 59-year-old woman, who had been menopausal for 5 years and had a huge abdominal mass underwent laparotomy; histopathology of the bulky tumor revealed the particular characteristics of a myxoid leiomyosarcoma. Immunohistochemically, Ki-67 contributed toward indicating a malignancy, whereas p53 and progesterone and estrogen receptors were negative. The level of serum Ca125 was high at diagnosis, within normal limits after the fifth cycle of chemotherapy, and subsequently increased again at recurrence. Sixteen months after surgery, the patient died of respiratory failure. Immunohistochemistry and Ca125 levels can aid the diagnosis, management, and prognosis of uterine myxoid leiomyosarcoma.

A huge abdominal mass mimicking ovarian cancer: p53-negative but aneuploid myxoid leiomyosarcoma of the uterus

Objective: Less than 20 myxoid leiomyosarcoma cases were reported in literature. Since, these tumors are very rare and may exhibit highly malignant behavior despite their low mitotic index, clinical course and optimum type of therapy of myxoid variant of leiomyosarcoma were not well understood. The goal of this report is to contribute the better understanding of this rare type of tumor. Methods: A 39-year-old woman presented with a huge abdominal cystic mass. Laparotomy was performed and frozen section diagnosis was low-grade uterine leiomyosarcoma. TAH-BSO, omentectomy, pelvic lymph node and peritoneal samplings were carried out. No chemotherapy was performed after surgical therapy. Results: Final histopathological diagnosis was uterine myxoid leiomyosarcoma. The tumor was p53-negative and had aneuploid DNA content. The patient tolerated well the operation and she is alive and free of disease after 24 months of primary surgical treatment. Conclusion: Uterine myxoid leiomyosarcoma may present a huge abdominal cystic mass and can be treated successfully with surgery alone.

Uterine myxoid leiomyosarcoma

Background: Uterine myxoid leiomyosarcoma is rare, has a poor prognosis, and must be distinguished from a uterine myoma with myxomatous change. Case: A 56-year-old woman with a history of epigastric pain and generalized abdominal swelling had a peritoneal cavity filled with tumor that originated from the posterior uterine wall. Chemotherapy and two operations for recurrence could not prevent fatal metastases to the vascular system. Histopathology showed a typical myxoid leiomyosarcoma. Conclusion: Although the patient’s prognosis was poor, no mitoses were observed, and the nuclear abnormalities were not sufficient to diagnose the tumor as malignant at the initial surgery. Whenever a uterine myoma with myxomatous change is found, the patient should be monitored carefully.

Uterine myxoid leiomyosarcoma within a leiomyoma

A case of myxoid leiomyosarcoma of the uterus arising in a leiomyoma is reported. Although the tumor showed very low mitotic activity ranging from zero to 2/10 HPF, the presence of infiltrative pattern of growth and a high MIB-1 index (60% of cells positive) established the diagnosis. Myxoid leiomyosarcoma may arise in leiomyoma.

Uterine myxoid leiomyosarcoma and CYVADIC-etoposide therapy

Objectives: To conduct a pathological characterization of rare uterine myxoid leiomyosarcoma (MLS) and establish an effective chemotherapy for metastasis after hysterectomy. Methods: Histological and immunohistochemical methods were used to characterize the type of uterine MLS in a 39-year-old female. For multiple lung metastases found 3 months after operation, CYVADIC (400 mg cyclophosphamide, 1 mg vincristine, 50 mg epirubicin and 400 mg dacarbazine per body for one course) and oral etoposide (50 mg/body per day for 2 weeks) were applied. Results: The rapidly enlarging uterine tumors exhibited gelatinous cut surfaces, a high count of 30 mitotic cells per 10 hpf and mucinous stroma, indicating a rare high-mitotic type of uterine MLS. The two-course CYVADIC-etoposide therapy was effective for disseminated pulmonary metastases. Conclusion: The present MLS was a rare high-mitotic type. CYVADIC-etoposide was an optional effective chemotherapy for lung metastasis in this case.