scholarly journals Case 1 − A case of gynecological PEComa

2017 ◽  
Vol 5 (1) ◽  
pp. 31-34
Author(s):  
Caterina Fontanella ◽  
Maria Luisa Carcangiu ◽  
Domenica Lorusso ◽  
Bruno Vincenti

Perivascular epithelioid cell tumors (PEComas) are a heterogeneous group of rare mesenchymal neoplasms composed of epithelioid cells which express melanocytic and myogenic markers, such as HMB-45, desmin and actin. In March 2013, we visited a postmenopausal 51-year-old woman with a suspected diagnosis of uterine PEComa diagnosed by a dilation and curettage of the lining of the uterus. After the histological revision of the formalin-fixed paraffin-embedded (FFPE) material by our expert pathologist, we confirmed the diagnosis and referred the patient for complete primary surgery. On November 2013, the patient underwent total abdominal hysterectomy and bilateral salpingo-oophorectomy with no macroscopically detectable residual cancer. However, in January 2014, due to voiding dysfunction and inconstant vaginal bleeding, the patient underwent magnetic resonance imaging (MRI) which documented multiple irregular lesions in the pelvis suspected as recurrent PEComa. Considering the early relapse of PEComa after optimal primary surgery, we suggested a systemic treatment with the combination of gemcitabine and docetaxel. For logistic reasons, the patients started the chemotherapy in her district hospital. After two cycles of chemotherapy the patient died due to treatment-related complications.

2004 ◽  
Vol 14 (5) ◽  
pp. 1036-1039 ◽  
Author(s):  
D. Fink ◽  
D. E. Marsden ◽  
L. Edwards ◽  
C. Camaris ◽  
N. F. Hacker

Malignant perivascular epithelioid cell tumor (PEComa) is an extremely rare mesenchymal neoplasm mostly composed of HMB-45-positive epithelioid cells with clear-to-eosinophilic cytoplasm, a propensity for perivascular distribution and a coexpression of smooth muscle markers. The uterus seems to be one of the most prevalent sites of involvement, although only 14 cases of uterine PEComa have been described. We report the case of a 51-year-old woman with a PEComa arising in the broad ligament. She was treated with total abdominal hysterectomy, bilateral salpingo-oophorectomy, omentectomy, and pelvic radiation, and remains without evidence of disease 15 months after diagnosis. This is, to the best of our knowledge, the first report of a malignant PEComa arising in the broad ligament. To correctly diagnose PEComa, an extensive immunohistochemical panel is essential. As PEComas can behave in an aggressive manner, careful follow-up is warranted.


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Miyoshi A ◽  
◽  
Ueda Y ◽  
Sato K ◽  
Kimura T ◽  
...  

Mullerian adenosarcoma of the endometrium in adolescent girls is extremely rare, with only fifteen cases under 20 years old having been reported to date. We describe here a new case of adolescent Mullerian adenosarcoma and provide an updated review of the previous literature on such rare tumors. Our 19-year-old case presented with a six-month history of prolonged menstruation. She had not yet had any sexual relationship. On gross examination, a fragile mass was seen in her vagina that bled easily. A 4.0×2.0 cm mass was visualized with Magnetic Resonance Imaging (MRI). The tumor seemed to slightly invade the myometrium of the uterine corpus. Transvaginal ultrasound sonography confirmed the presence of a 4.0 cm mass located in the cervix and vagina. The tumor biopsy was diagnosed as a Mullerian adenosarcoma of the endometrium. We performed a Total Abdominal Hysterectomy (TAH) and Bilateral Salpingectomy (BS). The post-surgical specimen was diagnosed as a pT1aNXM0 Mullerian adenosarcoma of the endometrium. The patient did not require adjuvant chemotherapy. She has been monitored every 3 months and has been without recurrence now for 28 months.


Cancers ◽  
2020 ◽  
Vol 12 (10) ◽  
pp. 2832 ◽  
Author(s):  
Yamini Krishna ◽  
Amelia Acha-Sagredo ◽  
Dorota Sabat-Pośpiech ◽  
Natalie Kipling ◽  
Kim Clarke ◽  
...  

Metastatic uveal melanoma (mUM) to the liver is incurable. Transcriptome profiling of 40 formalin-fixed paraffin-embedded mUM liver resections and 6 control liver specimens was undertaken. mUMs were assessed for morphology, nuclear BAP1 (nBAP1) expression, and their tumour microenvironments (TME) using an “immunoscore” (absent/altered/high) for tumour-infiltrating lymphocytes (TILs) and macrophages (TAMs). Transcriptomes were compared between mUM and control liver; intersegmental and intratumoural analyses were also undertaken. Most mUM were epithelioid cell-type (75%), amelanotic (55%), and nBAP1-ve (70%). They had intermediate (68%) or absent (15%) immunoscores for TILs and intermediate (53%) or high (45%) immunoscores for TAMs. M2-TAMs were dominant in the mUM-TME, with upregulated expression of ANXA1, CD74, CXCR4, MIF, STAT3, PLA2G6, and TGFB1. Compared to control liver, mUM showed significant (p < 0.01) upregulation of 10 genes: DUSP4, PRAME, CD44, IRF4/MUM1, BCL2, CD146/MCAM/MUC18, IGF1R, PNMA1, MFGE8/lactadherin, and LGALS3/Galectin-3. Protein expression of DUSP4, CD44, IRF4, BCL-2, CD146, and IGF1R was validated in all mUMs, whereas protein expression of PRAME was validated in 10% cases; LGALS3 stained TAMs, and MFGEF8 highlighted bile ducts only. Intersegmental mUMs show differing transcriptomes, whereas those within a single mUM were similar. Our results show that M2-TAMs dominate mUM-TME with upregulation of genes contributing to immunosuppression. mUM significantly overexpress genes with targetable signalling pathways, and yet these may differ between intersegmental lesions.


2013 ◽  
Vol 27 (1) ◽  
pp. 4
Author(s):  
Kemal Sarsmaz ◽  
Asli Goker ◽  
Naci Kemal Kuscu

Urinary retention is uncommon in reproductive age women. The frequent causes for obstructive retention are pelvic masses and a history of surgery. We report a case of intermittent urinary retention caused by a leiomyoma. A 47-year old patient with voiding difficulties for the past four months was referred to the gynecology outpatient clinic. She had urinary retention and had been catheterized several times for bladder emptying. She had been evaluated by the urology clinic and a cystoscopy was performed with normal findings. Magnetic resonance imaging (MRI) revealed a pelvic mass at the anterior uterine wall with a diameter of 10 cm. Her physical examination confirmed the MRI findings. Total abdominal hysterectomy was performed with a complete resolution of the patient’s complaints. Women with urinary retention should be consultated with a gynecologist in order to rule out pelvic masses.


2014 ◽  
Vol 2014 ◽  
pp. 1-4
Author(s):  
Shigeki Taga ◽  
Mari Sawada ◽  
Aya Nagai ◽  
Dan Yamamoto ◽  
Ryoji Hayase

Adenosarcoma is a rare tumor which consists of benign glandular epithelium and malignant mesenchymal component. Here we report a case of adenosarcoma of the uterine corpus.Case Presentation. A 59-year-old woman presented with vaginal bleeding and visited a local clinic. She had a uterine tumor pointed out and was referred to our hospital. Ultrasound scans revealed a large heterogeneous mass occupying the whole uterine cavity. Cytological test of endometrium was performed but the result was negative. A fractional endometrial curettage revealed no malignancy. Magnetic resonance imaging (MRI) revealed a heterogeneous solid tumor of 77 × 76 mm. Total abdominal hysterectomy with bilateral salpingo-oophorectomy and pelvic lymphadenectomy was performed. On gross examination, the tumor was arising from the uterine body and occupied the whole uterine cavity. Histopathological examination revealed phyllodes-like architecture on low magnification and periglandular cuffing of tumor cells. The lesion was confined to the uterus. Histopathological final diagnosis was adenosarcoma. Her postoperative course was uneventful and she was discharged without postoperative treatment and remains alive without disease 6 months after the surgery.


1994 ◽  
Vol 4 (3) ◽  
pp. 194-199 ◽  
Author(s):  
A. Ph. Makar ◽  
R. Holm ◽  
G. B. Kristensen ◽  
J. M. Nesland ◽  
C. G. TropÉ

The expression of c-erbB-2 oncogene has been studied in 74 formalin fixed paraffin embedded invasive epithelial ovarian malignancies using a monoclonal c-erbB-2 protein antibody. Only four (5.4%) tumors showed membrane immunostaining. FIGO stage, size of residual disease after primary surgery, histologic type and tumor grade, were identified by the log rank test as prognostic factors for survival. No difference in survival was observed between those with and without membrane staining (24 months probability of survival: 50% and 55.4% respectively). Our results did not show any adverse prognostic effect of c-erbB-2 protein expression in our patients.


Author(s):  
B Archana ◽  
Sandhya Sundaram ◽  
Lawrence D Cruze ◽  
KS Rajeshwari

Uterine sarcomas are relatively rare and account for only 1-3% of all malignancies in the Female Genital Tract (FGT). Adenosarcomas of the uterus are uncommon accounting for only 5% of uterine sarcomas. They are considered less recurrent and less metastatic. However, those with sarcomatous overgrowth and heterologous differentiation are very rare, considered aggressive and need to be differentiated histologically from other sarcomas that mimic the same clinically and histologically. In the index case, a 50-year-old female presented with symptoms of pain in the abdomen and bleeding per vagina. Magnetic Resonance Imaging (MRI) showed a large polypoid solid mass in the endometrium, suggestive of a sarcoma. Positron Emission Tomography (PET) scan showed an Fluorodeoxyglucose (FDG) avid lesion in the endometrial cavity (SUV max-13.33). Total abdominal hysterectomy and salpingooophorectomy was performed. Histology showed a biphasic tumour (benign glands and malignant mesenchymal component) with rhabdomyoblastic differentiation and sarcomatous overgrowth. Presence of rhabdoid cells raises the possibility of other sarcomas with these features making the diagnosis challenging. Immunohistochemistry (IHC) confirmed it to be a high grade adenosarcoma with sarcomatous overgrowth. Desmin positivity was noted in the rhabdoid cells. Majority cases of adenosarcoma are low grade, the index case was however high grade with presence of heterologous elements and sarcomatous overgrowth. Neither radiology nor clinical features can reliably differentiate adenosarcomas from other uterine sarcomas. A definitive diagnosis can be made by understanding the overlapping morphological features and correlate it with IHC to reliably differentiate it with other similar looking uterine sarcomas.


2012 ◽  
Vol 30 (15_suppl) ◽  
pp. 5032-5032
Author(s):  
Johanne Ingrid Weberpals ◽  
Jaime Snowdon ◽  
Olga Bougie ◽  
Xiao Zhang ◽  
Victor Tron ◽  
...  

5032 Background: Reliably predicting which LEC patients are most likely to recur is a challenge for the clinician with implications on adjuvant therapy. MiRNAs have been exploited for diagnosis and prognostication in a number of malignancies. We hypothesize that miRNA expression profiles differ in tumors from patients with recurrence compared to those without recurrence. Methods: The inclusion criteria for this study are informed consent, stage 1 disease, grade 1 or 2 tumors and endometrioid histology. RNA was extracted from formalin-fixed paraffin-embedded tissues and miRNA profiling was done using Agilent Human miRNA. Differentially expressed miRNAs were identified using GeneSpring GX software and the two groups were compared using the student t-test. Results: The expression levels of 866 miRNAs were determined from LEC patients with recurrence (n=15) and without recurrence (n=16). The mean follow-up interval was 61.5 months. The average age of cancer diagnosis for patients with and without recurrence was 60.2 (range 42-75) and 59.7 (range 44-86), respectively (p=0.91). Three of 15 patients with recurrence and 6 of 16 patients without recurrence received adjuvant brachytherapy following their primary surgery (p=0.43). 17 miRNAs were identified which can distinguish between the tumors with recurrence and those without recurrence (p<0.05). MiR-146a, miR-18a, miR-222, and miR-30a showed the highest fold change difference (>5 fold) in the tumors with recurrence compared to that did not recur. A decision tree prediction model for recurrent LEC was developed where a miRNA cutoff was used as a branch in the decision tree. This model identified those patients who were most likely to recur based on the expression of 4 dysregulated miRNAs (miR-222, miR-361-3p, miR-181c and miR-125b). Conclusions: These preliminary results show the miRNA expression profile differs among LEC and can be used to distinguish an aggressive sub-group. Should future validation studies confirm this result, this information would be valuable in the design of a biomarker study to help decide which patients would benefit most from extended adjuvant treatment.


2004 ◽  
Vol 14 (4) ◽  
pp. 694-696 ◽  
Author(s):  
K. Sakhel ◽  
A. Khalil ◽  
H. Kaspar ◽  
G. Azar ◽  
A. Mansour ◽  
...  

Placental site trophoblastic tumor (PSTT) is the rarest form of gestational trophoblastic diseases. We report a 40-year-old woman who presented initially after a year of secondary infertility with prolonged menstrual flow. Her last pregnancy ended in a suction dilation and curettage at 8 weeks' gestation for a missed abortion. A hysterosalpingogram revealed a solid 5 × 4.5 cm filling defect impinging on the endometrial cavity suggestive of a submucosal leiomyoma. A vaginal sonogram confirmed the findings. The patient underwent a laparotomy for excision of the 5-cm friable, necrotic mass that was performed by curetting. Pathologic examination of the specimen revealed sheets of intermediate trophoblastic cells characterized by large polyhedral cells and positive human placental lactogen staining consistent with PSTT. The patient eventually underwent total abdominal hysterectomy 4 weeks later with no evidence of residual tumor on histologic examination.


2021 ◽  
Vol 5 (1) ◽  
Author(s):  
Miyoshi A ◽  
◽  
Ueda Y ◽  
Sato K ◽  
Kimura T ◽  
...  

Mullerian adenosarcoma of the endometrium in adolescent girls is extremely rare, with only fifteen cases under 20 years old having been reported to date. We describe here a new case of adolescent Mullerian adenosarcoma and provide an updated review of the previous literature on such rare tumors. Our 19-year-old case presented with a six-month history of prolonged menstruation. She had not yet had any sexual relationship. On gross examination, a fragile mass was seen in her vagina that bled easily. A 4.0×2.0 cm mass was visualized with Magnetic Resonance Imaging (MRI). The tumor seemed to slightly invade the myometrium of the uterine corpus. Transvaginal ultrasound sonography confirmed the presence of a 4.0 cm mass located in the cervix and vagina. The tumor biopsy was diagnosed as a Mullerian adenosarcoma of the endometrium. We performed a Total Abdominal Hysterectomy (TAH) and Bilateral Salpingectomy (BS). The post-surgical specimen was diagnosed as a pT1aNXM0 Mullerian adenosarcoma of the endometrium. The patient did not require adjuvant chemotherapy. She has been monitored every 3 months and has been without recurrence now for 28 months.


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