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

Abstract

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.

Primary Fallopian Tube Mullerian Adenosarcoma With Sarcomatous Overgrowth and a Previously Unreported MEIS1-NCOA2 Gene Fusion

“…The majority of MAs arise in the uterine corpus, uterine cervix or the ovaries. A smaller subset arises from the fallopian tube and the peritoneum, and may be related to endometriosis (6)(7)(8). Because of the rarity of extrauterine MA, we herein report a case of MA arising in the fallopian tube, with sarcomatous overgrowth, diffuse peritoneal involvement and a MEIS1-NCOA2 gene fusion.…”

Primary ovarian endometrioid stromal sarcoma presenting with reno-ureteral colic

“…Distant metastatic lesions, if any, are usually preceded by intra-abdominal recurrences. A recurrence can occur as a low-grade or high-grade adenosarcoma or as a sarcoma of variable grade [ 12 , 13 ].…”

Epidemiology of adenosarcoma and the inverse probability of treatment weighting (IPTW) adjusted survival analysis of lymph node dissection in uterine adenosarcoma

“…[ 2 , 4 , 5 ] Adenosarcoma occasionally occurs in extra-uterine and extra-ovarian sites such as the peritoneum, retroperitoneum, bladder, liver or colon, and it is generally assumed that the biological behaviors of extra-uterine adenosarcoma is associated with endometriosis. [ 3 , 6 ] Uterine adenosarcoma accounts for 2% to 5% of all uterine sarcomas. [ 7 ]…”

“…[2,4,5] Adenosarcoma occasionally occurs in extra-uterine and extra-ovarian sites such as the peritoneum, retroperitoneum, bladder, liver or colon, and it is generally assumed that the biological behaviors of extra-uterine adenosarcoma is associated with endometriosis. [3,6] Uterine adenosarcoma accounts for 2% to 5% of all uterine sarcomas. [7] It is well known that sarcoma is a malignant tumor originating from mesenchymal tissue, and adenocarcinoma originated HH and ZW contributed equally to this study.…”