Uterine PEComa Research Articles

MTOR inhibitor in the treatment of TFE-positive advanced maligmnant PEComa of the uterus: a case report and literature review.

The pre- and intra-operative diagnoses of malignant uterine vascular perivascular epithelioid cell tumors (PEComas) can be challenging, for which the literature is limited. Some cases have been shown to have TSC gene mutations or rearrangements of the MiT factor family, resulting in variable responses to mTOR inhibitors. We report a case of a TFE-positive malignant PEComa of the uterus with pulmonary metastases that responded favorably to the mTOR inhibitor, everolimus. A 52-year-old female underwent a total hysterectomy 5 years ago for suspected sub-serosal or broad ligament fibroids. The intraoperative pathologic diagnosis was leiomyosarcoma of the uterus and the postoperative diagnosis was malignant PEComa of the uterus. The patient declined genetic testing and further treatment. In December 2020 the patient presented with a pelvic mass and underwent open abdominal mass resection and pelvic adhesiolysis. The pathologic findings confirmed recurrent malignant PEComa of the uterus. The pulmonary lesions gradually progressed during the follow-up period, so treatment with everolimus was initiated. Close follow-up evaluation for nearly 3 years showed disease remission without recurrence or progression. The patient described herein had a TFE-positive uterine malignant PEComa with lung metastasis and responded well to the mTOR inhibitor, everolimus. Close follow-up in the last 3 years showed remission without recurrence or progression.

Clinical and ultrasound features of uterine perivascular epithelioid cell tumors: case series and literature review.

To describe the clinical and ultrasonographic features of uterine perivascular epithelioid cell tumor (PEComa) using standardized terminology. This was a retrospective analysis of patients with uterine PEComa diagnosed and confirmed by pathology and immunohistochemistry at West China Second University Hospital, Sichuan University, Sichuan, China, between January 2010 and September 2023. The Morphological Uterus Sonographic Assessment (MUSA) consensus and the International Endometrial Tumor Analysis (IETA) consensus were utilized for the standardized description of the sonographic characteristics of uterine PEComa. We summarized the clinical and ultrasound features of uterine PEComa in cases from our center and those found in a review of the literature conducted using PubMed from 1 January 2013 to 30 September 2023 (inclusive). Five patients, aged 33-57 (median, 52) years with a total of six uterine PEComa lesions were included in our cohort. All cases had complete ultrasonographic and pathological images. None of the patients had a history of tuberous sclerosis complex. Two patients had malignant PEComa (one patient had two lesions) and three had benign PEComa, originating from the cervix, myometrium or uterine cavity. Patients presented with symptoms including increased vaginal discharge, vaginal bleeding and pelvic or abdominal pain. The three patients with benign PEComa underwent total hysterectomy and bilateral adnexectomy, tumor excision and conservative management, respectively, while both malignant cases underwent total hysterectomy and bilateral adnexectomy followed by chemotherapy. Regular follow-up (from 6 to 24 months) revealed recurrence in one case. Two lesions were misdiagnosed as uterine fibroids, two as cervical cancer, one as metastatic cervical cancer (with myometrial invasion) and one was indeterminate. Ultrasound examination showed that most lesions displayed regular round or ovoid shapes (66.7%), uniform echoes (66.7%) and hypoechogenicity (66.7%), with one (16.7%) malignant PEComa showing cystic areas and one (16.7%) benign PEComa showing punctate calcifications. All lesions lacked shadowing and the majority showed moderate to abundant vascularity (color score of 3-4, 83.3%). The color score was 2-4 in the periphery in 100% of cases and internally in 83.3% of cases. The three benign PEComas showed similar characteristics in vascular distribution, with scattered internal vessels and peripheral vessels exhibiting a circular pattern. The literature search identified 11 articles describing the ultrasonographic appearance of 18 cases of uterine PEComa, with similar characteristics to those in our cohort. The sonographic features of uterine PEComa include a uniform or non-uniform hypoechogenic mass, typically round or ovoid with regular margins, occasionally containing cystic areas or calcifications, lacking shadowing and often showing moderate to abundant vascularity. Although the preoperative ultrasound diagnosis of uterine PEComa remains challenging, particularly given the non-specific nature of the sonographic characteristics described here, dispersed intratumoral vessels and a peripheral circular vascular distribution may serve as diagnostic clues for uterine PEComa, but more cases are needed for confirmation. © 2024 International Society of Ultrasound in Obstetrics and Gynecology.

Malignant Perivascular epithelioid cell tumour of the uterus without TFE3 gene rearrangement: a case report

BackgroundPerivascular epithelioid cell tumours (PEComas) are soft tissue tumours. These neoplasms belong to the family of mesenchymal tumours, which include angiomyolipomas, clear-cell sugar tumours of the lung, and PEComas not otherwise specified (NOS). The probability of a perivascular epithelioid cell tumour (PEComa) occurring in the uterus is low, and the incidence, diagnosis, treatment, and outcomes of such tumours are still unclear.Case presentationA 51-year-old woman presented a 4-year history of natural menopause. An intrauterine mass was detected via ultrasound examination; the mass showed a tendency to increase but caused no symptoms. The levels of tumour markers were within the normal range. Pathological analysis of the curettage revealed perivascular epithelioid differentiation of the endometrial tumour. Consequently, a laparoscopic total hysterectomy with bilateral adnexectomy was performed. No distant metastasis was detected via whole-body positron emission computed tomography (PETCT) after the operation. Fluorescence in situ hybridization (FISH) revealed no TFE3 gene rearrangement. Next-generation sequencing of bone and soft tissue revealed negative TSC1/2 and TP53 expression. No recurrence or metastasis was observed during the 18-month follow-up period.ConclusionPEComa of the gynecologic tract is a rare and challenging entity. Diffuse HMB-45 expression, TSC alterations and TFE3 rearrangement are characteristic of uterine PEComas. Surgical resection is the first choice. Genetic testing is helpful for determining the nature of the mass and for choosing targeted therapy. Further research is needed to establish treatment protocols.

Abstract B010: Response to treatment with nab-sirolimus among patients with primary uterine tumors: A subgroup analysis from AMPECT

Abstract Introduction/objective: AMPECT (NCT02494570) is a pivotal study of nab-sirolimus in patients with malignant perivascular epithelioid cell tumors (PEComa). Malignant PEComa is an aggressive, rare sarcoma for which cytotoxic chemotherapies provide limited patient benefit. Inactivation of TSC1 or TSC2 tumor suppressor genes, upstream of mTORC1, is commonly associated with malignant PEComa; these mutations occurred in 56% of patients in AMPECT. nab-Sirolimus is an mTOR inhibitor (mTORi) that utilizes albumin-bound nanoparticle technology to drive higher intratumoral drug levels and more complete pathway suppression than conventional mTORis. nab-Sirolimus is approved in the USA for the treatment of advanced or metastatic malignant PEComa based on the primary analysis results of the AMPECT trial, which showed a confirmed overall response rate (ORR) of 39%, regardless of mutation status. We describe outcomes of a subset of patients with primary uterine PEComa, representing 23% of efficacy-evaluable patients in AMPECT. Methods: AMPECT was an open-label, multicenter, phase 2 registration study in mTORi-naïve adult patients (≥18 years old) with a histologically confirmed diagnosis of malignant PEComa and Eastern Cooperative Oncology Group performance status score ≤1. Patients received nab-sirolimus 100 mg/m2 intravenously on days 1 and 8 of a 21-day cycle until progression or unacceptable toxicity. The primary endpoint was ORR by independent radiology review. Key secondary endpoints included time to response, duration of response (DOR), and safety. Results: A total of 7 patients with uterine PEComa were treated with nab-sirolimus in AMPECT and were evaluable for both safety and efficacy. Mean ± SD age was 60.0 ± 13.4 years, and 71% (5/7) of patients were White. ORR was 43% (3/7 patients); all 3 were confirmed partial responses and were seen in patients with tumors with somatic inactivating alterations in TSC1 or TSC2. Two (29%) patients had best response of stable disease. For the 3 responders, time to response was 1.4, 1.4, and 1.5 months, and DOR was 5.6, 36.0+, and 39.7 months. All 7 patients had ≥1 treatment-related adverse event (TRAE; grade 3, 43%); no grade ≥4 or serious TRAEs occurred. Conclusions: Rates of response to treatment and safety findings in patients with primary uterine PEComa treated with nab-sirolimus in AMPECT were consistent with those previously reported in the overall patient population. Responses were rapid and durable. Notably, all responders had TSC1 or TSC2 alterations; however, there were too few patients in this subset to draw conclusions regarding efficacy in tumors without these alterations. nab-Sirolimus is being further evaluated in a tumor-agnostic trial of patients with TSC1 or TSC2 inactivating alterations (PRECISION I; NCT05103358). Citation Format: Vinod Ravi, Mark A. Dickson, Kristin Ganjoo, Li Ding, Anita N. Schmid, Norma A. Palma, Willis H. Navarro, Andrew J. Wagner. Response to treatment with nab-sirolimus among patients with primary uterine tumors: A subgroup analysis from AMPECT [abstract]. In: Proceedings of the AACR Special Conference on Endometrial Cancer: Transforming Care through Science; 2023 Nov 16-18; Boston, Massachusetts. Philadelphia (PA): AACR; Clin Cancer Res 2024;30(5_Suppl):Abstract nr B010.

Systematic analysis of perivascular epithelioid cell neoplasms in the female reproductive tract: a comprehensive review.

Perivascular epithelioid cell neoplasms (PEComas) are rare mesenchymal lesions, with gynecological PEComas accounting for just over a quarter of cases. Limited reports exist on gynecological PEComa, primarily treated with surgery; adjuvant therapy is considered in high-risk cases. This systematic review aims to summarize the origin and clinical, pathological and molecular characteristics of uterine PEComa, focusing on treatment options for gynecological PEComa. A comprehensive PubMed review of gynecological PEComa reports was conducted. A detailed examination of the literature ensured a thorough understanding. Gynecological PEComa diagnosis relies on histology and immunology. Despite therapy controversies, surgery remains the mainstay. Adjuvant therapy efficacy in high-risk cases is uncertain. mTOR inhibitors are the first line; alternative treatments, including angiogenesis and aromatase inhibitors, should be considered.

Sarcomas With RAD51B Fusions Are Associated With a Heterogeneous Phenotype

RAD51B-rearranged sarcomas are rare neoplasms that exhibit a heterogeneous morphology. To date, 6 cases have been reported, all involving the uterus, including 4 perivascular epithelioid cell tumors (PEComas) and 2 leiomyosarcomas (LMS). In this study, we describe the morphologic, immunohistochemical, and molecular features of 8 additional sarcomas with RAD51B rearrangement, including the first extrauterine example. All patients were women with a median age of 57 years at presentation. Seven tumors originated in the uterus, and one in the lower extremity soft tissue, with a median tumor size of 12 cm. Histologically, 4 tumors showed predominantly spindle cell morphology with eosinophilic fibrillary cytoplasm, with or without nuclear pleomorphism, whereas 2 tumors exhibited pleomorphic epithelioid cells, featuring clear to eosinophilic, granular cytoplasm. Two neoplasms exhibited undifferentiated cytomorphology, including one with uniform small blue round cells. All tumors showed high-grade cytologic atypia and high mitotic activity (median: 30/10 high-power fields), whereas coagulative necrosis was noted in 6 cases and lymphovascular invasion in 2. By immunohistochemistry, 2 showed myoid and melanocytic markers in keeping with PEComa, whereas 4 cases were only positive for smooth muscle markers consistent with LMS (including 3 myxoid). The remaining 2 cases had a nonspecific immunoprofile. Five cases tested by targeted RNA sequencing (Archer FusionPlex, Illumina TruSight) showed different fusion partners (HMGA2, PDDC1, and CEP170). RAD51B rearrangements were identified by FISH in the remaining 3 cases. Targeted DNA sequencing in 2 cases was negative for TSC gene alterations. Clinical outcome, available in 5 patients (median follow-up, 19 months), revealed 3 local recurrences, 2 lung metastases, and 4 deaths due to disease. Our results expand the spectrum of sarcomas with RAD51B fusions, demonstrating variable clinical presentations, morphologic spectrum, and fusion partners. These tumors have a predilection for a uterine location, with either LMS, PEComa, or undifferentiated phenotypes, and are associated with an aggressive clinical course.

Diagnostic des sarcomes utérins et tumeurs mésenchymateuses utérines rares à potentiel de malignité. Référentiels du Groupe Sarcome Français et des Tumeurs Rares Gynécologiques

The landscape of uterine sarcomas is becoming more complex with the description of new entities associated with recurrent driver molecular alterations. Uterine sarcomas, in analogy with soft tissue sarcomas, are distinguished into complex genomic and simple genomic sarcomas. Leiomyosarcomas and undifferentiated uterine sarcomas belong to complex genomic sarcomas group. Low-grade and high-grade endometrial stromal sarcomas, other rare tumors associated with fusion transcripts (such as NTRK, PDGFB, ALK, RET ROS1) and SMARCA4-deficient uterine sarcoma are considered simple genomic sarcomas. The most common uterine sarcoma are first leiomyosarcoma and secondly endometrial stromal sarcomas. Three different histological subtypes of leiomyosarcoma (fusiform, myxoid, epithelioid) are identified, myxoid and epithelioid leiomyosarcoma being more aggressive than fusiform leiomyosarcoma. The distinction between low-grade and high-grade endometrial stromal sarcoma is primarily morphological and immunohistochemical and the detection of fusion transcripts can help the diagnosis. Uterine PEComa is a rare tumor, which is distinguished into borderline and malignant, according to a risk assessment algorithm. Embryonal rhabdomyosarcoma of the uterine cervix is more common in children but can also occur in adult women. Embryonal rhabdomyosarcoma of the uterine cervix is almost always DICER1 mutated, unlike that of the vagina which is wild-type DICER1, and adenosarcoma which can be DICER1 mutated but with less frequency. Among the emerging entities, sarcomas associated with fusion transcripts involving the NTRK, ALK, PDGFB genes benefit from targeted therapy. The integration of molecular data with histology and clinical data allows better identification of uterine sarcomas in order to better treat them.

EP26.01: Imaging review of five cases of uterine PEComas

EP26.01: Imaging review of five cases of uterine PEComas

Response to treatment with nab-sirolimus among patients with primary uterine PEComa: A sub analysis from AMPECT (1289)

Response to treatment with nab-sirolimus among patients with primary uterine PEComa: A sub analysis from AMPECT (1289)

Contrasting Outcomes in Two Cases of Uterine PEComa

Perivascular epithelioid cell tumours of the uterus are rare neoplasms with only few cases described in the literature. Ongoing contribution to the literature is important in order to understand the natural history for the diagnosis and management challenges for PEComa. We present two contrasting cases of PEComa who presented with postmenopausal bleeding, were diagnosed on endometrial biopsy and had surgical treatment. The first patient following treatment has been disease free for four years while on regular follow-ups. The second patient had aggressive disease developed early recurrence requiring chemotherapy and died in 17 months from diagnosis.PEComa is a rare tumour with usually good outcomes, where surgery itself is adequate treatment. There is not much evidence available currently on the use of chemotherapy in rare cases, such as our second case, where PEComa presents aggressively. In this article, we elaborate two cases of contrasting outcomes with one presentation being aggressive requiring chemotherapy. We evaluated the current evidence on use of chemotherapy in rare cases.

Malignant perivascular epithelioid cell tumor (PEComa) of the uterus

BackgroundPerivascular epithelioid cell tumors (PEComas) of the uterus is a rare type of mesenchymal tumors associated with myelomelanocytic differentiation and distinctive histological appearances. So far, the reported cases of uterine PEComas are usually benign. Documented malignant cases with aggressive behavior appear to be less common.Case presentationWe report a 37-year-old female who received abdominal hysterectomy for uterine tumor in a local hospital. She was diagnosed with uterine leiomyosarcoma and referred to Hubei Cancer Hospital. Her histological slides were reviewed and immunohistochemical staining for specific markers of epithelial, melanocytic, myoid and some others were analyzed. The pathologic diagnosis was malignant uterine PEComa. Systematic imaging of the patient further revealed an abdominal para-aortic mass. She received pelvic and para-aortic lymph node dissection. Postoperative histology revealed para-aortic lymph nodal metastasis of malignant uterine PEComa. She received 8 cycles of chemotherapy after surgery. The chemotherapy regiment was epirubicin plus ifosfamide The patient is free of recurrence and metastasis 6 years after surgical resection.ConclusionUterine PEComas are indistinguishable from other uterine tumors such as leiomyoma and leiomyosarcoma before pathologic diagnosis could be made. For patients with malignant uterine PEComas, removal of both primary lesions and metastatic foci, if any, needs to be attempted. Postoperative chemotherapy or radiotherapy should also be considered in patients with distant metastases or positive lymph nodes.

Uterine PEComas: correlation between melanocytic marker expression and TSC alterations/TFE3 fusions

Uterine PEComas often present a diagnostic challenge as they share morphological and immunohistochemical features with smooth muscle tumors. Herein we evaluated a series of 19 uterine PEComas to compare the degree of melanocytic marker expression with their molecular profile. Patients ranged from 32–77 (median 48) years, with six tumors classified as malignant based on the modified gynecologic-specific prognostic algorithm. All patients with malignant PEComas were alive with disease or dead of disease at last follow-up, while all those of uncertain malignant potential were alive and well (median follow-up, 47 months).Seventeen of 19 (89%) PEComas harbored either a TSC1 or TSC2 alteration. One of the two remaining tumors showed a TFE3 rearrangement, but the other lacked alterations in all genes evaluated. All showed at least focal (usually strong) positivity for HMB-45, with 15/19 (79%) having >50% expression, while the tumor lacking TSC or TFE3 alterations was strongly positive in 10% of cells. Melan-A and MiTF were each positive in 15/19 (79%) tumors, but staining extent and intensity were much more variable than HMB-45. Five of six (83%) malignant PEComas also harbored alterations in TP53, ATRX, or RB1, findings not identified in any tumors of uncertain malignant potential. One malignant PEComa was microsatellite-unstable/mismatch repair protein-deficient.In summary, TSC alterations/TFE3 fusions and diffuse (>50%) HMB-45 expression are characteristic of uterine PEComas. In morphologically ambiguous mesenchymal neoplasms with myomelanocytic differentiation, especially those with metastatic or recurrent disease, next-generation sequencing is recommended to evaluate for TSC alterations; as such, patients can be eligible for targeted therapy.

Rapid and Durable Response With Nab-Sirolimus After Everolimus Failure in a Patient With Perivascular Epithelioid Cell Tumors (PEComas) of the Uterus

Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors with a natural history ranging from indolent benign lesions to ones with an aggressive clinical course including distant metastases. Recent reports have suggested that mTOR inhibitor sirolimus and related drugs show some benefit in non-tuberous sclerosis complex PEComas. However, therapeutic options for patients who progress on sirolimus are very limited. We describe a patient with metastatic uterine PEComa, who progressed on mTOR inhibitor everolimus but had a rapid and durable response to nab-sirolimus.

VEGFR Inhibitors for Uterine Metastatic Perivascular Epithelioid Tumors (PEComa) Resistant to mTOR Inhibitors. A Case Report and Review of Literature.

Uterine perivascular epithelioid cell tumors (PEComas) are rare neoplasms. PI3K/AKT/mTOR pathway upregulation is critical for their pathogenesis and is often associated with TSC1/TSC2 inactivation. Although first line mTOR inhibitors are an effective treatment, metastatic PEComas eventually progress. A 53-year-old woman presented a 4-month history of post-menopausal vaginal bleeding. Clinical and radiological examination detected a uterine mass and a single S1 bone lesion. The patient underwent a radical hysterectomy and bone biopsy. The anatomopathological evaluation concluded to an oligo-metastatic uterine PEComa. The tumor harbored a heterozygous deletion of 9q34 that contains the TSC1 gene. Concerning the primary lesion, the resection was complete and the single bone metastasis was treated with radiotherapy. Three months later, the patient presented bone, lung and subcutaneous metastatic progression. An everolimus and denosumab treatment was initiated. After 2 years of treatment, a clinically significant bone, lung and subcutaneous progression was detected. Following a literature review of the possible therapeutic options, we initiated a second line treatment by pazopanib. This treatment resulted in regression of the subcutaneous lesions and stability of lung and bone metastases. In this challenging, rare setting, our report suggests single agent, anti-angiogenic, tyrosine kinase inhibitor to be effective as second line treatment of metastatic uterine PEComa progressing on mTOR inhibitors.

Malignant perivascular epithelioid cell tumor (PEComa) of uterus: A case report with literature review

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm involving various organs. Approximately 70 cases of gynecologic PEComas have been reported in the literature. Due to the paucity of cases, there is no definite treatment regimen established. We describe disease progression in a case of uterine PEComa with pulmonary metastasis in a 37-year-old woman who had been treated with surgery and various regimens of adjuvant treatment including vincristine, ifosfamide, cisplatin, sirolimus, doxorubicin, olaratumab, pazopanib and dacarbazine. Adequate management and prognosis still seem inconclusive and further case reports with randomized trials are needed to delineate the nature of this neoplasm.

PNL2: A Useful Adjunct Biomarker to HMB45 in the Diagnosis of Uterine Perivascular Epithelioid Cell Tumor (PEComa).

Perivascular epithelioid cell tumors (PEComa) are rare neoplasms characterized by co-expression of melanocytic and muscle markers. HMB45 and Melan-A are used to confirm a PEComa diagnosis; however, both are often focally expressed and sensitivity for Melan-A is low. PNL2 is a reliable biomarker for epithelioid melanoma and renal angiomyolipoma/PEComa. The objective of this study was to determine PNL2 utility in diagnosing uterine PEComas as well as distinguishing PEComas from uterine smooth muscle tumors (SMTs). Twenty-one uterine PEComas and 45 SMTs were analyzed for PNL2; a subset was also stained for HMB45, Melan-A, Cathepsin-K, Desmin, and h-Caldesmon. Cases were scored as negative (0), focal (<10% of tumor cells), or patchy to diffusely positive (>10% of tumor cells). PEComas were positive for PNL2, HMB45, and Melan-A in 86%, 100%, and 57% of cases, respectively. In PEComas, PNL2 was patchy to diffusely positive more frequently (10/18, 56%) than Melan-A (4/12, 33%). In contrast, 2 of 45 (4%) SMTs were focally PNL2 positive; HMB45 was focally positive in 4 SMTs (11%) and all were negative for Melan-A. Desmin and h-Caldesmon were positive in 90% and 57% of PEComas, and 91% and 82% of SMTs. Cathepsin-K was positive in 100% of PEComas and 93% of SMTs. PNL2 is a useful biomarker for the diagnosis of uterine PEComa, with comparable sensitivity and specificity to HMB45. In contrast, PNL2 stains more PEComas when compared with Melan-A. Cathepsin-K, Desmin, and h-Caldesmon are of little utility for distinguishing PEComas and SMTs; however, lack of Cathepsin-K argues against PEComa. These results suggest that PNL2 should be used in conjunction with HMB45 in the diagnosis of PEComa of the uterine corpus.

MR findings of uterine PEComa in patients with tuberous sclerosis: report of two cases

Tuberous sclerosis complex (TSC), a rare autosomal dominant neurocutaneous disorder, is characterized by the presence of benign congenital tumors in multiple organs. Neoplasms with perivascular epithelioid cell differentiation (PEComas), including angiomyolipoma (AML) and lymphangioleiomyomatosis (LAM), can occur in association with TSC. This report describes two cases of uterine PEComas presenting characteristic MR imaging features reflecting pathological findings. From MR images, both cases showed single or multiple large, irregularly shaped or lobulated hemorrhagic lesions within the myometrium. They differed from typical adenomyotic cysts in their large size and irregular margins. Histopathologic analysis revealed that the hemorrhage was caused by adenomyosis and tumor cells that proliferated in surrounding stroma of the hemorrhagic lesions, compatible with PEComas. Microscopic observation revealed an infiltrative growth pattern of PEComas, with small nodules formed. The tumor lesions, however, were difficult to detect on MR images. The myometrium showed normal appearance on both T1-weighted and T2-weighted images in both cases. We speculate that PEComas may infiltrate extensively into the myometrium even when the myometrium shows almost normal radiologic appearance.

Integrated management of Pecoma of uterus with Ayurveda

Integrative complimentary therapy with Ayurveda and yoga in cancer practice has wide potential to prevent reduce and manage the cancer as well as complications of cancer and therapy too Chemotherapy Radiotherapy Surgery Hormonal therapyMethods 56yr female diagnosed case of uterine Pecoma surgically operated TAHBSORPLN receiving oral chemo came with complaints of chronic Anaemia exhaustion weight loss and abdominal swelling associated with pain on. Patient was treated considering her prakruti vikruti and clinical presentation. Initially she was started with Amalaki swarasa later patient was given Haratala Bhasma 125mgday early morning in empty stomach for 1 month. After 1-month Patient received Bhallataka Lavana 14th teaspoon with food for 1 month. Dhatri Loha 1tabtwice a day Hinguvachadi gulika 1tabthrice a day Kalyanaka kshaara 14th tsftwice a day for 5 months continued under regular follow up along with suitable yoga practices.Results Subjective Complaints like exhaustion weakness bloating pain and tenderness comparatively reduced during the course of therapy. Over all CT whole abdomen on 14.06.2018 showed significant reduction in the lesion from 1810cm14.4cm to 12.36.8. 12.3cm APTRCC with minimal Ascites which showed good control of disease.Conclusion Integrated therapy can increase recovery as well as improvement in clinical and pathological condition which was evident in current case study.

1613PD - mTOR inhibitors in uterine and extra-uterine malignant PEComas: A multicenter international case series retrospective analysis

1613PD - mTOR inhibitors in uterine and extra-uterine malignant PEComas: A multicenter international case series retrospective analysis

Perivascular epithelioid cell tumor (PEComa) presents an endometrial polyp pattern: Case report and literature review

Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor composed of histologically and immunohistochemically distinctive perivascular epithelioid cells [1, 2], which can arise in a wide array of anatomic locations, and is characterized by its myelomelanocytic phenotype and unpredictable natural history. To date, less than 80 cases of uterine PEComa have been reported in the literature [3]. Although almost all of these cases show a mural or subserosal localization. Here, we report a rare pattern of uterine PEComa which present as polypoid lesions mimicking an endometrial polyp in a 47-year-old Chinese woman.