Adenosarcoma ; pathology ; surgery ; Adolescent ; Female ; Humans ; Uterine Cervical Neoplasms ; pathology ; surgery

Adenosarcoma/surgery* ; Female ; Heart Neoplasms ; Humans ; Neoplasm Metastasis ; Uterine Cervical Neoplasms ; Uterine Neoplasms/surgery*

Adenofibroma ; pathology ; Adenosarcoma ; metabolism ; pathology ; surgery ; Aged ; Diagnosis, Differential ; Endometrial Neoplasms ; pathology ; Female ; Follow-Up Studies ; Humans ; Hysterectomy ; Keratin-7 ; metabolism ; Middle Aged ; Mucin-1 ; metabolism ; Polyps ; pathology ; Postoperative Period ; Preoperative Period ; Uterine Neoplasms ; metabolism ; pathology ; surgery

OBJECTIVETo investigate the clinicopathologic features, diagnosis, treatment and prognosis of uterine mullerian adenosarcoma.

METHODSThe clinicopathological data of 9 cases of uterine mullerian adenosarcoma in PUMC hospital from January 2003 to February 2009 were retrospectively analyzed.

RESULTSThere were 6 uterine endometrial adenosarcomas and 3 cervical adenosarcomas. The main clinical manifestations were abnormal vaginal bleeding and pelvic pain. Physical examination showed cervical/vaginal mass, enlarged uterus or pelvic mass. The adenosarcoma was characterized by benign or atypical-appearing neoplastic glands within a sarcomatous stroma. This stroma could appear as periglandular cuffs or intraglandular polypoid projections of increased cellular structure. The primary diagnostic rate was 66.7% and the most common clinical stage was stage I (7/9). All patients received surgical treatment and seven had postoperative chemotherapy, radiotherapy or hormone therapy. Conservation of unilateral ovary or bilateral ovaries was performed in 5 cases. Three patients underwent local excision, which resulted in the preservation of reproductive function. During the follow-up, 2 cases of uterine endometrial adenosarcoma recurred. One patient of clinical stage III containing sarcomatous overgrowth died from recurrence 13 months after surgery. The other one recurred 2 years after local excision of the tumor in the uterine cavity and she remained healthy since hysterectomy.

CONCLUSIONUterine mullerian adenosarcoma is a rare tumor without specific clinical symptoms and signs. The diagnosis depends on pathomorphologic examination. The tumors show low malignant potential and the vast majority are at early stage. Surgical excision is the main treatment strategy with a good prognosis in the early stage disease with complete removal of tumors. The prognosis is poor in advanced adenosarcoma with sarcomatous overgrowth. Due to the relatively high rate of recurrence, long-term follow-up is recommended.

Adenosarcoma ; drug therapy ; pathology ; surgery ; Adolescent ; Adult ; Antineoplastic Combined Chemotherapy Protocols ; therapeutic use ; Chemotherapy, Adjuvant ; Cisplatin ; therapeutic use ; Endometrial Neoplasms ; drug therapy ; pathology ; surgery ; Etoposide ; therapeutic use ; Female ; Follow-Up Studies ; Humans ; Hysterectomy ; methods ; Ifosfamide ; therapeutic use ; Middle Aged ; Neoplasm Recurrence, Local ; Neoplasm Staging ; Retrospective Studies ; Uterine Cervical Neoplasms ; drug therapy ; pathology ; surgery ; Uterine Neoplasms ; drug therapy ; pathology ; surgery ; Young Adult

OBJECTIVETo study the clinicopathologic features and differential diagnosis of recurrent Müllerian adenofibroma (MAF) of the uterus.

METHODSClinicopathologic information of 7 cases of recurrent MAF of uterus was retrieved from January 1992 to April 2006 and compared with 12 cases of MAF without recurrence and 14 cases of low-grade Müllerian adenosarcoma (MAS). EnVision immunohistochemistry of estrogen receptor (ER), progesterone receptor (PR), smooth muscle actin (SMA), CD10, Ki-67 and p53 were performed in all cases.

RESULTSAll cases of recurrent MAF of the uterus were polypoid, lobulated, and broad based mass arising from the corpus or cervix. Microscopically, the tumor consisted of benign epithelial and mesenchymal components with low mitotic activity ( ≤ 1/10 HPF). The clinical and pathologic features of 3 recurrent tumors were similar to their primary tumors, while 4 cases of recurrent tumor presented with focally higher cellularity and mitotic activity, meeting the diagnostic criteria of adenosarcoma. The stromal expression patterns of ER, PR, SMA and p53 in recurrent MAF were similar to those of clinically benign MAF and low-grade MAS. Negative or focally positive stromal cell expression of CD10 was seen infrequently in recurrent MAF (1/7) and clinically benign MAF (1/12). In contrast, a moderate to strong CD10 staining was frequently seen in MAS (9/14, P < 0.05). The difference of Ki-67-labeling index between MAF and MAS did not reach a statistical significance (P > 0.05). Ki-67-labeling index increased in areas of periglandular stromal cuffing as compared with interglandular areas in all MAS cases, but it was not observed in either recurrent MAF or clinically benign MAF cases.

CONCLUSIONSRecurrent MAF may be associated with aggressive behavior. It is difficult to distinguish MAF from low-grade MAS. CD10 and Ki-67 staining pattern in stromal cells may be helpful for the differential diagnosis.

Adenofibroma ; metabolism ; pathology ; surgery ; Adenosarcoma ; metabolism ; pathology ; Adolescent ; Adult ; Aged ; Aged, 80 and over ; Biomarkers, Tumor ; metabolism ; Diagnosis, Differential ; Female ; Humans ; Hysterectomy ; methods ; Ki-67 Antigen ; metabolism ; Middle Aged ; Neoplasm Grading ; Neoplasm Recurrence, Local ; Neprilysin ; metabolism ; Survival Rate ; Uterine Neoplasms ; metabolism ; pathology ; surgery ; Young Adult