Extrauterine adenosarcoma arising in omental endometriosis: Rare site of occurrence of a rare tumor

Kar, Rakhee; Verma, Surendra Kumar; Dasari, Papa; Sylvia, Mary Theresa

doi:10.4103/0377-4929.142720

Cited by 4 publications

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“…AS was treated by surgical resection in 38/41 (92.7%) patients: 5/38 (13.2%) patients underwent partial resection, and 33/38 (86.8%) underwent total resection. Of the 38 patients who received surgical treatment, 18 (47.4%) underwent resection of only the tumour [5abc, 7, 8, 9, 11, 13, 18, 19d, 20, 22, 23, 24, 30, 34, 36, 37]; four (10.5%) underwent tumour resection, hysterectomy and bilateral salpingo-oophorectomy [ 14 , 28 , 32 , 38 ]; and 16 (42.1%) underwent extensive surgery involving other organs such as the bowel [12, 15, 19a, 19b, 19c, 25, 29, 31, 35] and liver [ 17 ].…”

Section: Resultsmentioning

confidence: 99%

“…In 13/41 (31.7%) patients menopausal status was not reported. Moreover, 17/41 (41.5%) [5a–c, 6, 10, 11, 13,16, 17, 19c, 21, 22, 25, 27, 29, 30, 39] patients were in postmenopausal stage, 11/41 (26.8%) patients were at premenopausal stage [ 4 , 8 , 9 , 12 , 14 , 18 , 24 , 31 , 36 – 38 ] and 4/11 (36.4%) received bilateral salpingo-oophorectomy during AS treatment [ 8 , 14 , 31 , 38 ].…”

Section: Resultsmentioning

confidence: 99%

“…Overall, 6/25 (24%) patients with endometriosis showed sarcomatous overgrowth [ 22 , 24 , 27 , 33 , 38 , 39 ]: 13/25 (52%) were only surgically treated [5c-10-17-19abc-21-29-30-32-33-36-37], 10/25 (40%) were treated with surgery and adjuvant therapy [11–15–22-23-24-25-26-31-38-39], 1/25 (4%) was treated with neoadjuvant chemotherapy and surgery [ 27 ], and 1/25 (4%) was treated with chemotherapy and hormonal therapy without surgery [ 16 ].…”

Section: Resultsmentioning

confidence: 99%

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Primary extra-uterine and extra-ovarian mullerian adenosarcoma: case report and literature review

et al. 2018

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BackgroundExtra-uterine mullerian adenosarcomas have varying biological behaviours depending on the presence of endometriosis or sarcomatous overgrowth. These behaviours manifest according to the tumours’ histological characteristics and sites of origin. The best treatment and oncologic outcome have not been clarified because only a few cases of extra-uterine and extra-ovarian adenosarcoma have been described in the literature. Here, we report a case of primary peritoneal adenosarcoma with sarcomatous overgrowth and review all reported cases of adenosarcomas arising outside of the uterus and outside the ovaries to identify the best treatment options and clarify outcomes.Case presentationA 79-year-old woman was referred to our Department with an abdominal mass resembling a fibroid with a haemorrhage. Her gynaecological history was negative. A transvaginal and transabdominal ultrasound examination revealed a multicystic mass resembling an ovarian tumour arising from the pelvis and extending up to the abdomen. At laparotomy a peritoneal mass arising from Douglas peritoneum was resected. The uterus and adnexa appeared normal, and a supra-cervical hysterectomy with bilateral salpingo-oophorectomy was performed. No macroscopic residual disease was present. Final pathology diagnosed a malignant peripheral nerve sheath tumors with divergent differentiation. Four weeks later a new, multicystic mass was found. Due to the progressive poor condition, the patient died four months after diagnosis. Histological slides were reviewed by external expert pathologists and the final diagnosis was of extra-genital adenosarcoma with sarcomatous overgrowth. Furthermore, we also collected and analysed articles written in English regarding extra-uterine and extra-ovarian adenosarcomas published between January 1974 and October 2016. PubMed was used as a database for this search. Clinical and pathological characteristics, treatments and outcomes were assessed.ConclusionsOnly 41 cases has been reported in literature. Previous endometriosis and sarcomatous overgrowth showed an inverse effect on prognosis. Endometriosis was confirmed to have a positive effect on disease free survival Complete surgical resection is the mainstay of treatment. A worldwide registry is urgently required to collect data to standardize treatment and to obtain reliable data on prognosis.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

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Primary extra-uterine and extra-ovarian mullerian adenosarcoma: case report and literature review

et al. 2018

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“…6,9 In the literature, among the risk factors described, the majority of adenosarcomas are associated with endometriosis. [13][14][15][16][17][18][19][20][21][22][23][24] Symptoms may vary according to location. In uterine adenosarcoma, the most common presenting symptom is abnormal vaginal bleeding, but some patients present pelvic pain, abdominal mass or vaginal discharge.…”

Section: Discussionmentioning

confidence: 99%

“…Outside the uterine cavity, the main locations of the cancer described are the ovary, peritoneum, pelvis and, in patients who underwent hysterectomy, the vagina. 14,[17][18][19][20][21][22][23][24][25] Other sites where extragenital Müllerian adenosarcoma may be found are the rectum and vaginalrectum septum. 13,15,16 In 2001, Hirakawa et al 24 have interestingly reported for the first time the presence of tumor cells in the ascitic fluid in a patient presenting adenosarcoma of the ovary.…”

Section: Discussionmentioning

confidence: 99%

Imaging Description of Extragenital Müllerian Adenosarcoma: A Case Report

Mone

Iannone

2018

Rev Bras Ginecol Obstet

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Müllerian adenosarcoma is a very rare gynecological disease, comprising 5% of uterine sarcomas. Extragenital localizations are even rarer. We report a very interesting case of a 27-year-old woman complaining of pelvic pain, with a subsequent diagnosis of extragenital Müllerian adenosarcoma. This is the first case reported in the literature with a complete and wide imaging description. Even if rare, Müllerian adenosarcoma should be hypothesized in case of young female patients presenting with suspicious pelvic mass.

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Uterine Adenosarcoma: a Review

et al. 2016

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Adenosarcomas are rare malignancies of the female genital tract, accounting for approximately 5 % of uterine sarcomas. Occasionally, adenosarcoma occurs in the ovaries or in extra-uterine tissue, which may be related to endometriosis. These tumors are characterized by benign epithelial elements and a malignant mesenchymal component. Pathologic diagnosis is dependent on the identification of the characteristic morphologic features. The most common immunohistochemical markers for adenosarcoma are CD10 and WT1, but these are not specific. The most frequent presenting symptom is abnormal uterine bleeding. The majority of patients present with stage I disease, with a 5-year overall survival of 60 to 80 %. Survival is influenced by the presence of myometrial invasion, sarcomatous overgrowth, lymphovascular invasion, necrosis, and the presence of heterologous elements including rhabdomyoblastic differentiation. Patients with sarcomatous overgrowth have significantly increased risk of recurrence 23 versus 77 % and decreased 5-year overall survival 50 to 60 %. Standard of care treatment is total hysterectomy with bilateral salpingo-oophorectomy without lymphadenectomy, as the incidence of lymph node metastasis is rare. Retrospective data does not support the use of adjuvant pelvic radiotherapy in uterine adenosarcomas as no survival benefit is seen. Insufficient data exists to recommend routinely neoadjuvant or adjuvant chemotherapy for uterine adenosarcomas. Limited evidence exists for the role of hormonal therapy in uterine adenosarcomas. The PIK3/AKT/PTEN pathway is mutated in ∼70 % of adenosarcomas, and this may represent a possible therapeutic target. This article reviews the current state of knowledge concerning uterine adenosarcoma and discusses the management of this rare tumor.

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Extrauterine adenosarcoma arising in omental endometriosis: Rare site of occurrence of a rare tumor

Cited by 4 publications

References 0 publications

Primary extra-uterine and extra-ovarian mullerian adenosarcoma: case report and literature review

Primary extra-uterine and extra-ovarian mullerian adenosarcoma: case report and literature review

Imaging Description of Extragenital Müllerian Adenosarcoma: A Case Report

Uterine Adenosarcoma: a Review

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