Primary retroperitoneal mucinous cystic neoplasm of borderline malignancy with KRAS and GNAS co-mutation: a case report.

Primary retroperitoneal mucinous cystic neoplasms are rare retroperitoneal tumors, which are histologically similar to mucinous cystic neoplasms of the ovaries. Only 31 cases of primary retroperitoneal mucinous cystic neoplasm with borderline malignancy (PRMCN-BM) have been reported (26 in women and five in men). We describe an additional male patient with PRMCN-BM. A 39-year-old man presented to our hospital with back pain. Twelve years earlier, he had undergone an orchiectomy for a germ cell tumor. Computed tomography showed a 6.9- × 4.4-cm cystic mass in the left pararenal space. Laparoscopic mass excision was performed, and a unilocular cystic mass was found in the pararenal space near the lower pole of the left kidney. A histopathological examination showed a cyst lined by atypical mucinous intestinal epithelium without stromal invasion. Targeted next-generation sequencing identified two hotspot mutations, with one each in the KRAS and GNAS genes. Outpatient follow-up 10 months after surgery showed no evidence of tumor recurrence. PRMCNs are extremely rare retroperitoneal neoplasms, especially in men. These neoplasms are rarely considered in the differential diagnosis of retroperitoneal masses, and their preoperative diagnosis is difficult. Evaluation of additional patients is required to better determine the prognosis of PRMCNs and the optimal postoperative follow-up.

A case of paraovarian tumor of borderline malignancy with decrease of apparent diffusion coefficient value and marked 18F-fluorodeoxyglucose accumulation.

Para-ovarian cysts are occasionally encountered in clinical practice; however, malignant tumors derived from them are rare. Due to its rarity, the characteristic imaging findings of para-ovarian tumors with borderline malignancy (PTBM) are largely unknown. Herein, we report a case of PTBM, along with imaging findings. A 37-year-old woman came to our department with a suspected malignant adnexal tumor. Pelvic contrast-enhanced magnetic resonance imaging (MRI) revealed a solid part within the cystic tumor with a decrease in the apparent diffusion coefficient (ADC) value (1.16 × 10-3 mm2/s). We also performed Positron Emission Tomography-MRI and showed a strong accumulation of 18F-fluorodeoxyglucose (FDG) in the solid part (SUVmax = 14.8). In addition, the tumor appeared to develop independently of the ovary. Because tumor was derived from para-ovarian cyst, we suspected PTBM preoperatively and planned fertility sparing treatment. Pathological examination revealed a serous borderline tumor and PTBM was confirmed. PTBM can have unique imaging characteristics, including a low ADC value and high FDG accumulation. When a tumor appears to develop from para-ovarian cysts, borderline malignancy can be suspected, even if imaging findings suggest malignant potential.

Endometrial Cancer in a Patient With Didelphys Uterus and a History of Renal Cancer: A Case Report and Literature Review.

Müllerian duct anomalies (MDAs) concurrent with endometrial cancer are exceptionally rare, with only a few documented cases. Here, we present a case of endometrial cancer in both horns of a didelphys uterus in a 54-year-old woman with a history of renal cancer, who underwent left radical nephrectomy and left salpingo-oophorectomy. The patient sought medical evaluation due to postmenopausal vaginal bleeding. Hysteroscopy with dilation and curettage revealed the presence of two cervixes and two endometrial cavities, with pathology results indicating endometrioid adenocarcinoma (G1). Preoperative MRI staging confirmed the diagnosis of a double cervix and uterus. Subsequently, an open abdominal hysterectomy and a right salpingo-oophorectomy were performed, revealing a didelphys uterus (International Federation of Gynaecology and Obstetrics 2018, stage IA). This manuscript aims to explore the potential correlation between renal and endometrial malignancies in the presence of MDAs.

Management of borderline ovarian tumors: A tertiary referral center experience in Egypt.

Background: In this retrospective study, we discuss our experience as a large tertiary referral center in Egypt in the management and follow-up of borderline tumors. Patients and methods: This is a retrospective cohort study where all patients diagnosed with a borderline ovarian tumor at Oncology Center Mansoura University from November 2014 to June 2020 were included. Demographics, preoperative, operative, postoperative, pathologic, and oncologic follow-up data were retrieved from a prospectively maintained electronic database. The included patients were followed until April 2022. Results: We included 27 patients with borderline ovarian tumors. The mean age of the study patients was 47.67 ± 16.39 years. The median CA 125 was 33 (6-304 U/ml). Frozen section examination was utilized in 13 patients (48.14%), where a diagnosis of borderline ovarian tumors was revealed in 8 patients. Recurrence was reported in one patient with serous type after approximately 26 months. The most common pathological type in our cohort was the mucinous borderline type reported in 14 patients (51.9%), followed by the serous type reported in 11 patients (40.7%), and the seromucinous type in 1 patient only. Patients with mucinous borderline type were significantly younger (40.083 ± 18.47 vs. 53.73 ± 11.91 years, p = 0.028). Interestingly, Cancer Antigen 125 levels were significantly higher in mucinous than serous and seromucinous types [67(16-304) vs. 20(6-294.6) U/ml, p = 0.027]. On the other hand, the radiological tumor size of serous and seromucinous types was larger than that of the mucinous type [23(19-31) cm vs. 8(5-20) cm, p = 0.001]. Over a median follow-up period of 58.66 (54.16-63.16) months, only one postoperative mortality was reported, while only one recurrence was reported. Conclusion: Borderline ovarian tumors still represent a dilemma either in diagnosis or management. A frozen section examination could help to reach a preliminary diagnosis. Total abdominal hysterectomy and bilateral salpingo-oophorectomy are the cornerstone of surgical management; however, fertility-sparing surgery could be a valid option for women desiring fertility.

Treatment strategy for pediatric giant mucinous cystadenoma: A case report.

Because of their rarity, the treatment strategy for pediatric ovarian epithelial tumors is controversial, especially for a giant cystadenoma. We report the largest mucinous cystadenoma (MCA) case in the pediatric literature thus far. A 12-year-old girl had abdominal distention and visited our hospital. She had a multilocular cyst with some protuberance on the inside and high values of CA 19-9 and CA-125. We diagnosed her with a left MCA and performed a left oophorectomy. The tumor was the stage IA borderline malignant MCA and weighed 11.8 kg. Five years have passed, the patient has not experienced recurrence or metastasis. The resection of giant tumors can affect respiration and circulation. However, pre- or intra-operative drainage may lead to dissemination and adhesion. When we treat pediatric giant ovarian epithelial tumors, we must understand the findings that suggest the possibility of malignancy to decide appropriately as to whether drainage should be performed.

Psoas/iliacus muscle invasion from mucinous appendiceal neoplasm. Radiologic appearance and outcome of treatment in 3 patients.

BACKGROUND: Mucinous appendiceal neoplasms have a pattern of metastases that is different from the other gastrointestinal cancers. The first site for cancer dissemination is the peritoneal space surrounding the primary tumor and this is followed by increasingly extensive peritoneal spread. Invasion of the psoas and iliacus muscle is an unusual phenomenon. METHOD: From a prospective database of appendiceal mucinous neoplasms treated by cytoreductive surgery (CRS) and perioperative hyperthermic chemotherapy (HIPEC), patients with psoas muscle invasion were reviewed. Their clinical features and treatments were tabulated. RESULTS: Three patients with ages 33, 60, and 63 were identified. Two patients had disease progression into the psoas muscle 33 and 95 months after CRS plus HIPEC. One had dissecting mucinous tumor into psoas, iliacus and quadratus lumborum muscle at the time of diagnosis of the appendiceal mucinous neoplasm. All three survived at least five years from their initial treatment. CONCLUSION: Despite the fact that mucinous tumor invasion was outside the peritoneal cavity, long term benefit from psoas muscle resection with a mucinous appendiceal neoplasm is possible and resection possibly with HIPEC should be considered.

Anaplastic carcinoma in ovarian seromucinous cystic tumor of borderline malignancy.

BACKGROUND: The mortality rate of ovarian cancer is the highest among all gynecological malignancies in Japan. Ovarian tumors are classified as benign, borderline malignant, or malignant. Anticipating the histological subtype with imaging only is often difficult because of several histological subtypes of epithelial ovarian tumors (such as serous, mucinous, endometrioid, clear cell, and Brenner tumors). In addition, the majority of mucinous tumors in the ovary are metastatic. Furthermore, mucinous tumors belong to one of the two different subclasses (i.e., intestinal and seromucinous types). Ovarian seromucinous cystic tumors of borderline malignancy are infrequent and only rarely coexist with other malignant tumors. CASE PRESENTATION: We have reported a 53-year-old Japanese woman with anaplastic carcinoma in an ovarian seromucinous cystic tumor of borderline malignancy. Her MRI and CT analysis revealed an ovarian tumor with a mural nodule, ascites, and peritoneal dissemination. Enhanced MRI revealed that the mural nodule was enhanced. Enhanced CT analysis revealed that the lymph nodes were not swollen. Intriguingly, the mural nodule crossed the cyst wall into the cavity and onto the surface. Her laboratory data revealed high serum CA 125 level. Cumulatively, these results suggested ovarian malignancy. The patient underwent hysterectomy with bilateral salpingo-oophorectomy, omentectomy, and resection of the disseminated lesions. Lymph node biopsy was omitted because of the suggestion of enhanced CT image findings and palpation during surgery. Her postoperative specimen examination determined FIGO at least stage IIIB, and accordingly, adjuvant chemotherapy was prescribed. After 3 years of the operation, the patient is presently alive without clinical tumor recurrences. CONCLUSION: Imaging studies with pathognomonic findings contributed to ovarian cancer diagnosis in this case. To the best of our knowledge, this is the first study in English literature to report detailed classification of mucinous borderline malignancy, seromucinous cystic, and anaplastic carcinoma in an ovarian seromucinous cystic tumor of borderline malignancy.

Primary retroperitoneal mucinous cystic tumors with borderline malignancy: a case report and literature review.

Primary retroperitoneal mucinous cystic tumors with borderline malignancy are rarely encountered. To date, only 12 cases have been reported in the literature. In this report, we present an additional case. A 65-year-old nulliparous woman complained of abdominal fullness. Her medical history included a hysterectomy and a single salpingo-oophorectomy performed 25 years prior to the present event. Physical examination revealed a large cystic mass in the abdomen and pelvis. During laparotomy, a cystic tumor measuring 21×14 cm in size was observed in the left retroperitoneal space. The tumor was resected, and the final diagnosis was primary retroperitoneal mucinous cystic cancer with borderline malignancy.

Sonographic findings of an ovarian serous surface papillary borderline tumor.

Sonographic findings of a serous surface papillary borderline tumor of the ovary have rarely been reported in the English literature. Here, we describe a case of serous surface papillary borderline tumor, which was depicted on gray-scale and Doppler ultrasonography as smoothly lobulated and polypoid heterogeneous echoic bilateral adnexal masses encased or surrounded by what was presumed to be normal-appearing ovarian follicles with increased vascular flow.

Pseudomyxoma peritonei.

Pseudomyxoma peritonei (PMP) is an uncommon "borderline malignancy" generally arising from a perforated appendiceal epithelial tumour. Optimal treatment involves a combination of cytoreductive surgery (CRS) with heated intraperitoneal chemotherapy (HIPEC). Controversy persists regarding the pathological classification and its prognostic value. Computed tomography scanning is the optimal preoperative staging technique. Tumour marker elevations correlate with worse prognosis and increased recurrence rates. Following CRS with HIPEC, 5-year survival ranges from 62.5% to 100% for low grade, and 0%-65% for high grade disease. Treatment related morbidity and mortality ranges from 12 to 67.6%, and 0 to 9%, respectively. Surgery and HIPEC are the optimal treatment for PMP which is at best a "borderline" peritoneal malignancy.

A case of borderline clear cell fibroadenoma / 대한산부인과학회지

Clear cell tumor of borderline malignancy has been reported to account for 5 to 8% of all clear cell neoplasms, and <1% of borderline malignancies of the ovary. Borderline clear cell fibroadenoma with or without microinvasion mostly occurs in women with middle age, and the presenting symptoms associated with this tumor are non-specific. The clinico-pathologic features of borderline clear cell fibroadenoma are not well known because of its rarity. Furthermore, there is no consensus for treating the disease. Thus, we report a case of borderline clear cell fibroadenoma presenting as ovarian fibroadenoma with a review of literature.

A case of parovarian tumor of borderline malignancy / 부인종양

Parovarian cysts, generally known as hydatid cysts of Morgagni, are small round cysts attached by a pedicle to the fimbriated end of the tube. Due to the rarity of this lesion, there are controversies concerning the origin, clinical behavior, treatment and prognosis of these tumors. Parovarian borderline malignancy mostly occur in young women, main complaints are abdominal enlargement and pelvic pain. we experienced one case of parovarian borderline malignancy and report this case with a brief review of literature.

Accuracy of frozen section diagnosis for ovarian tumors according to histologic type and malignant potential / 부인종양

OBJECTIVE: The purpose of this was to evaluate accuracy of frozen section diagnosis for ovarian tumors according to histologic type and malignant potential. METHODS: We compared the frozen section and final diagnosis of patients with ovarian tumors from April 2001 to April 2006. Of these 1138 cases, 628 cases (55.2%) were epithelial ovarian tumors. Benign, borderline, and malignant epithelial tumors were 380 (60.5%), 87 (13.9%), and 161 (25.6%) cases. The accuracy of frozen section diagnosis was analyzed according to histologic type and malignancy potential. RESULTS: The overall accuracy of frozen section diagnosis was 93.9%. The accuracy for benign, borderline, and malignant tumors were 93%, 92%, and 98%, respectively. The accuracy of frozen section diagnosis was significantly low in mucinous tumors and borderline malignant tumors. However the borderline malignancy was the only independent factor associated with the inaccuracy of frozen section diagnosis (OR: 12.2, 95% CI: 6.5-23.1). The sensitivity for immature teratoma was as low as 63.6%. CONCLUSION: Our data shows that the accuracy is low in mucinous tumors, borderline tumors, and immature teratomas and the borderline malignancy is independent factor associated with inaccuracy of frozen section diagnosis.

A case of paraovarian tumor of boderline malignance / 대한산부인과학회지

Paraovarian cysts arise in the mesosalpinx, between the ovarian hilum and the fallopian tube. Clinical significance of paraovarian tumors is considerably less frequent than ovarian tumors, and malignant paraovarian lesions are exceedingly rare. Paraovarian carcinomas or borderline malignancy mostly occur in young women. Abdominal enlargement and pelvic pain are the usual complaints. Usually they have a capsule, are unilateral and are connected to the broad ligament. Little is known about the biological behavior of the paraovarian borderline malignancies, since these lesions are so rare. The appropriate therapy for this unusual lesion have not been fully defined. We had experienced a case of papillary serous adenocarcinoma of borderline malignancy and report this case with a brief review of literature.

A case of primary parovarian cystadenocarcinoma of borderline malignancy / 대한산부인과학회지

Parovarian tumors may be of mesothelial, mesonephric (Wolffian), or paramesonephric (Mullerian) origin. An estimated 10% of adnexal masses are parovarian cysts, most commonly mesothelial or paramesonephric in origin. Benign neoplasms such as cystadenomas may occasionally develop in parovarian cysts. Malignancy has been reported in 2.0% to 2.8% of parovarian cystic masses, but it seems to be even less frequent in masses smaller than 5 cm. The histologic appearance of the tumor is identical with that of tumors of ovarian origin. However, their similarity of biologic behavior is uncertain. The appropriate therapy for this unusual lesion has not been fully defined, since a minimal amount of follow-up data is available on the few cases which have been reported. We had experienced a case of papillary serous cystadenocarcinoma of borderline malignancy arising from a parovarian paramesonephric cyst and report this case with a brief review of literature.

Mucinous Borderline tumor associated with Mature Cystic Teratoma of Ovary / 부인종양

OBJECTIVE: To evaluate the clinicopathological characteristics of mucinous borderline tumor associated with mature cystic teratoma and the origin of its cell type. METHODS: Five patients who were diagnosed as mucinous borderline tumor associated with mature cystic teratoma and treated in Samsung Cheil Hospital from January 1996 to September 2004 were analyzed. In addition MEDLINE, other data bases in English as well as in Korean revealed two more cases. We analyzed the result with patient's characteristics, preoperative symptoms, mass sizes, serum CA-125, operative procedure, stages, recurrence and survival period. Histological slides of the specimens were analysed for expression of cytokeratin (CK)-7 and cytokeratin (CK)-20 by immunohistochemical staining to know the origin of the cell. RESULTS: The mean age was 45 years (25-61 years). Preoperative symptoms were palpable abdominal mass, abdominal pain and urinary frequency. The mean level of CA-125 was 46.05 U/mL and the mean tumor size was 16.75 cm (8.8-22.0 cm). Post surgical FIGO staging was; stage Ia in 6 cases, stage Ic in 1 case. Four cases were CK7(-)/CK20(+), reflecting gastrointestinal origin, and also showed pattern of pseudomyxoma peritoneii. Mean survival period was 32.7 months (6-67 months) without evidence of recurrence, except one case. CONCLUSION: Our findings suggest that mucinous borderline tumor associated with mature cystic teratoma, is diagnosed at relatively young age and early stage, associated with good prognosis.

A Case of Parovarian Serous Cystadenocarcinoma of Borderline Malignancy / 대한산부인과학회잡지

Although parovarian cysts constitute 10-20% of all adnexal masses, malignant parovarian tumors are extremely rare. Due to the rarity of this lesion, there are controversies concerning the origin, clinical behavior, and prognosis of these tumors. Currently, it is suggested that these paovarian tumors should be treated similarly to their ovarian counterparts. We experienced one case of parovarian serous cystadenocarcinoma of borderline malignancy, and report this with a brief review of the literatures.

A Case of Ovarian Mucinous Cystadenoma, Borderline Malignancy in Pediatric Patient

Ovarian tumors of an epithelial origin in pediatric age are uncommon and mucinous cystadenoma, borderline malignancy is extremely rare. We experienced a case of ovarian mucinous cystadenoma, borderline malignancy in a 14-year-old girl. She was admitted due to huge abdominal distension for 2 months. Her past history and family history were normal. The physical examination was normal except tense and distended abdomen. All laboratory findings were normal. On abdominal ultrasonography and CT scan, a huge cystic tumor filled with fluid and inner multiseptation was noted. Unilateral salphingo- oophorectomy was done and pathologic findings were compatible with ovarian borderline malignancy of mucionous cystadenoma. The postoperative course was uneventful.

A Serous Papillary Cystadenoma of Low Malignant Potential in Paratesticular Tissue

The serous papillary cystadenoma of low malignant potential in the paratesticular tissue is a tumor characterized by its resemblance to ovarian serous papillary tumor of low malignant potential. Several possible origins have been proposed but it is thought to originate from the Mullerian-type epithelium just like ovarian serous tumor of low grade malignancy. Because of the rarity of this tumor, the natural history and treatment have not been established. Herein we report on a serous papillary cystadenoma arising in the paratesticular tissue of a 12 year old . To our knowledge, this is the first reported case in the Korean literature. The size of this cystic tumor in this patient was 0.9x0.9x0.8 cm and had a sponge-like cut surface. The papillary structure was lined by both ciliated and nonciliated secretory cells, the same cellular structure as the salpinx. Areas with just a few mitoses and stratification of epithelial cells were noted, but invasion into the paratesticular tissue was absent, suggesting borderline malignancy. He is doing well after 5 months follow up. Prognosis of this scrotal tumor is thought to be better than the ovarian tumor of similar histology due to a better possibility of early detection.