Pseudomixoma peritoneal primario de origen ovárico / Primary pseudomyxoma peritonei originated from ovaries

Resumen ANTECEDENTES: El pseudomixoma peritoneal es muy raro; se caracterizada por ascitis mucinosa e implantes peritoneales relacionados con la rotura y diseminación del contenido de un tumor mucinoso. En 80 al 90% de los casos el tumor primario es apendicular y el ovario es una localización realmente excepcional. CASO CLÍNICO: Paciente de 49 años que acudió a consulta debido a un dolor abdominal. En las pruebas de imagen se visualizó una tumoración anexial izquierda y se advirtieron hallazgos sugerentes de pseudomixoma peritoneal. En la cirugía, la masa ovárica se objetivó parcialmente fragmentada, con el apéndice aumentado de tamaño y extensos implantes peritoneales; además de gran cantidad de mucina libre en la cavidad peritoneal. El análisis anatomopatológico determinó la existencia de un adenocarcinoma mucinoso en el ovario afectado, con inmunohistoquímica positiva para CK7+ y CK20+, múltiples implantes de mucina y el apéndice sin daño. Por lo anterior se diagnosticó: pseudomixoma peritoneal de origen ovárico. Luego de dos intervenciones quirúrgicas no se consiguió la citorreducción completa. La paciente permaneció estable durante siete años, momento en el que fue evidente el avance de los síntomas de la enfermedad, circunstancia que la condujo a la muerte. CONCLUSIÓN: Determinar el origen de un pseudomixoma peritoneal sigue siendo un reto pues, con frecuencia, tanto el apéndice como los ovarios se afectan simultáneamente. Por ello, la apendicectomía y la exploración bilateral de los ovarios deben ser prácticas de rutina. El análisis extenso de las muestras y la inmunohistoquímica pueden facilitar la catalogación de estos infrecuentes tumores.

Abstract BACKGROUND: Pseudomyxoma peritonei is very rare; it is characterized by mucinous ascites and peritoneal implants related to rupture and dissemination of the contents of a mucinous tumor. In 80 to 90% of cases the primary tumor is appendicular and the ovary is a truly exceptional location. CLINICALCASE: A 49 year old female patient presented for consultation due to abdominal pain. Imaging tests showed a left adnexal tumor and findings suggestive of pseudomyxoma peritonei. At surgery, the ovarian mass was partially fragmented, with an enlarged appendix and extensive peritoneal implants; in addition to a large amount of free mucin in the peritoneal cavity. The anatomopathological analysis determined the existence of a mucinous adenocarcinoma in the affected ovary, with positive immunohistochemistry for CK7+ and CK20+, multiple mucin implants and an undamaged appendix. Therefore, a diagnosis was made: pseudomyxoma peritoneum of ovarian origin. After two surgical interventions she did not achieve complete cytoreduction. The patient remained stable for seven years, at which time the symptoms of the disease became evident and led to her death. CONCLUSION: Determining the origin of a pseudomyxoma peritonei remains a challenge as often both the appendix and ovaries are affected simultaneously. Therefore, appendectomy and bilateral ovarian exploration should be routine practice. Extensive specimen analysis and immunohistochemistry can facilitate cataloging of these infrequent tumors.

Primary retroperitoneal mucinous cystadenocarcinoma: a rare surgico-pathological entity

Ovarian cancer (OC) is the seventh most common cancer for females in the world. Epithelial OC is the most predominant pathologic subtype (85%-90%), with five major histotypes- serous, mucinous, endometrioid, brenner and clear cell tumour. Each have three pathological subtypes ie benign, borderline and malignant. Mucinous cystadenomas and carcinomas of the ovary are well-established and common tumors. Primary retroperitoneal mucinous cystadenomas and carcinomas are being very rare and histopathogenesis of which is still uncertain. Most pathologists suggest their origin through mucinous metaplasia in a pre-existing mesothelium-lined cyst. An accurate preoperative diagnosis of these tumors is challenging due to lack of established effective diagnostic measures. Hereby presenting a 50-year-old woman, who visited to the hospital with abdominal distension and discomfort since two months. Sonography and computed tomography scans were performed and showed large predominantly cystic lesion arising from right adnexa. Patient underwent exploratory laparotomy for removal of the tumor. Histology and immunohistochemistry revealed primary retroperitoneal mucinous cystadenocarcinoma.

A rare case presentation of large mucinous ovarian cystadenocarcinoma in a young female

Here authors report a case of large ovarian mucinous cystadenocarcinoma in a young female complicating young pregnancy. Ovarian mucinous adenocarcinoma is a rare ovarian tumour that arises from the surface epithelium of the ovary. A 25-year young female, 9 days post-partum presented to the hospital with complain of abdominal distension. USG finding suggesting large solid cystic mass 36 × 14 cm arising from pelvis extending up to epigastrium. MRI pelvis evident of heterogenous hyperintense solid cystic mass lesion giving bunch of grapes appearance with size 24.5 × 25 × 11.5 cms seen in intra peritoneal space extending cranially up to epigastrium and caudally into pelvis giving anterior displacement of uterus.  And posterior displacement of bowel loops. A large part of lesion is cystic with solid component with multiple internal echoes.

Detection of gene mutation in pancreatic mucinous cystadenocarcinoma and its clinical significance / 中国肿瘤生物治疗杂志

@# Objective： ：To detect the distribution of gene mutations in pancreatic mucinous cystadenocarcinoma (PMCC) by highthroughput sequencing and to explore its clinical significance. Methods: Four cases of paraffin-embedded cancer tissues and paracancerous tissues from PMCC patients, who underwent surgical resection from January 2012 to December 2016, received NGS (next generation sequencing) examination using Illumina Hiseq 2500 platform. The characteristics of gene mutation in PMCC patients were analyzed with sequencing results and clinicopathological data. Results: Seven significantly mutated genes (SMGs) were detected in all four PMCC samples, namely KRAS, AHNAK2, MUC16, MUC17, MUC19, MUC3A and MUC4. Twenty-four SMGs were detected in 3 of the 4 samples, namely ADAMTS9, ALDH3B1, CARD14, CSMD3, MKI67, OR1N2, PKHD1, PLCE1, RTL1, SIGLEC12, CCDC168, CEP295, CUBN, DST, HRNR, LAMA5, OR10G4, OR2T4, PLEKHG4B, RP1L1, SLC15A5, SVEP1, TAS1R1 and TNRC18. KRAS-driven gene mutations were detected in all 4 samples, including K12 hot spot mutation in 3 cases and D33E non-hot spot mutation in 1 case. Conclusion: The high mutation of KRAS and MUC family in PMCC may be a potential target and biomarker for precise treatment of PMCC.

Histological Patterns of Ovarian Neoplasms – A Five Year Experience in North-East India

Ovary is one of the common sites of neoplasm in females. They manifest in wide spectrum of clinical, morphological and histological features. Ovary is the second most common site of primary malignancy in female genital tract.

Establishment of pancreatic mucinous cystadenocarcinoma cell line MCC1 with stable overexpression of miR-224 / 中国肿瘤生物治疗杂志

@#Objective: To construct a hsa-microRNA-224(miR-224) lentiviral expression vector and to establish pancreatic mucinous cystadenocarcinoma MCC1 cell line with stable miR-224 over-expression. Methods: Pri-miR-224 gene fragment was designed and amplified by quantitative real-time polymerase chain reaction (qRT-PCR), and then loaded into GV369 lentiviral vectors (GV369-miR224) by gene recombination technology. GV369-miR-224 lentivrial expression vectors were then identified by PCR and DNA sequencing. The GV369-miR-224 vector fluid was then used to infect pancreatic mucinous cystadenocarcinoma MCC1 cell line to establish the MCC1 cell line stably over-expressing miR-224. The transfection efficiency of GV369-NC and GV369-miR-224 was observed under fluorescence microscopy; and the expression levels of miR-224 in MCC1, GV369-miR-224-MCC1 and GV369-NC-MCC1 cell lines were detected by RT-PCR. Results: The GV369-miR-224 lentiviral vectors were successfully constructed. GV369-miR-224-MCC1 and GV369-NC-MCC1 cells all emit green fluorescence under the fluorescence microscope. The expression level of miR-224 in GV369miR-224-MCC1 cell group was significantly higher than that in negative control GV369-miR-224-MCC1 group and blank control MCC1 cell group (23.45±1.94， 1.46±0.1 and 2.11±0.38, P<0.01), however, there was no significant difference between the two control groups (P>0.05). Conclusion: A pancreatic mucinous cystadenocarcinoma MCC1 cell line with stable miR-224 over-expression was successfully established, and this will provide a new cell model for exploring the function and pathogenesis of miR-224 in pancreatic mucinous cystadenocarcinoma.

Radiologic Findings of Primary Mucinous Cystadenocarcinoma of the Breast: A Report of Two Cases and a Literature Review / 한국유방암학회지

Primary mucinous cystadenocarcinoma (MCA) of the breast is a rare but pathologically distinct breast tumor. There have been some case reports on primary MCA of the breast; however, they have all focused on pathologic findings. Here, we report the radiologic findings of two cases of MCA along with a review of the literature. Breast MCA shows a circumscribed mass with some calcifications on mammography, an intracystic solid mass without increased vascularity or a vascular stalk on ultrasound, and a heterogeneously enhancing mass within a rim-enhancing cyst with intermediate signal intensity on T2-weighted magnetic resonance imaging. These radiologic findings and the presence of mucin in the percutaneous biopsy specimen should suggest the possibility of MCA in the differential diagnosis of a breast tumor.

Spontaneous Rupture of Pancreatic Mucinous Cystadenocarcinoma: A Case Report and Review of Literature / 대한췌담도학회지

The pancreatic cystic lesions are known to be incidentally found up to 10-15% of patients undergoing cross-sectional imaging. And the prevalence of mucinous cystic neoplasm which has malignant potential is known to be up to 25% of all pancreatic cystic neoplasm in South Korea. The symptoms included abdominal pain, palpable mass, weight loss, loss of appetite, jaundice, asymptomatic and etc. However, spontaneous rupture of pancreatic mucinous cystadenocarcinoma (MCAC) is an extremely rare complication. Here we report a case of spontaneous rupture of pancreatic MCAC in a 72-year-old male with review of the literature. To the best of our knowledge, this is the first ruptured case of pancreatic MCAC in male patient.

A Case Report of Partial Nephrectomy of Mucinous Cystadenocarcinoma in Kidney and Its Literature Review / Journal of the Korean Cancer Association, 대한암학회지

Mucinous cystadenocarcinoma (MC) of the kidney is a rare epithelial tumor originating from the renal pelvic urothelium and few study cases have been reported. Because of the rarity of these tumors and their unknown histogenesis, its diagnosis is difficult until surgical exploration. We report here on a 55-year-old man referred to the urology department from the hepatology department because of a cystic renal mass measuring approximately 5 cm in size, which was detected incidentally under ultrasonography during the routine examination of liver. The renal mass was finally diagnosed as MC originating from kidney after partial nephrectomy and the patient still showed no evidence of recurrence until 12 months postoperatively. This is the first report on a case of renal MC in a patient who underwent partial nephrectomy. The aim of this report is to present our unusual case of MC and also review the previous literature on the pathological and radiological aspects of MC of kidney.

Prognostic significance of NAD (P) H-quinone oxidoreductase 1 overex-pression in ovarian mucinous cystadenocarcinoma / 中国病理生理杂志

AIM: To investigate the significance of NAD (P)H-quinone oxidoreductase 1 (NQO1) protein overexpression for prognostic evaluation of ovarian mucinous cystadenocarcinoma .METHODS:NQO1 protein was detected in 162 cases of ovarian mucinous cystadenocarcinoma , 35 cases of ovarian mucinous cystadenoma and 29 samples of normal ovarian epithelial tissues by the method of EnVision immunohistochemical staining .The correlation between high expression of NQO1 protein and clinicopathological features of ovarian mucinous cystadenocarcinoma was also evaluated .Overall sur-vival and disease-free survival rates of ovarian mucinous cystadenocarcinoma patients were calculated by Kaplan -Meier method.RESULTS:The positive rate and strongly positive rate of NQO 1 protein were 85.8%and 64.2%in ovarian mu-cinous cystadenocarcinoma , respectively , which are significantly higher than those in ovarian mucinous cystadenoma , and normal ovarian epithelial tissues ( P <0.01 ) .NQO1 expression was significantly correlated with the histological grade (P<0.05) and clinical stage (P<0.01) of ovarian mucinous cystadenocarcinoma .Kaplan-Meier survival analysis showed that the overall survival rate and disease-free survival rate were significantly higher in ovarian mucinous cystadenocarcinoma patients with high NQO1 expression than those with low NQO1 expression (P<0.01).CONCLUSION:NQO1 expression is closely correlated with the progression and prognosis of the patients with ovarian mucinous cystadenocarcinoma .High ex-pression of NQO1 protein may be used as an important indicator for the patients with poor prognosis of ovarian mucinous cystadenocarcinoma .

CT diagnosis of mucinous cystadenocarcinoma of appendix / 实用放射学杂志

Objective To explore the CT characteristics of mucinous cystadenocarcinoma of appendix.Methods The CT findings of 5 patients with mucinous cystadenocarcinoma of appendix proved by surgery and pathology were analyzed retrospectively,and the corresponding literatures were also reviewed.Results All lesions in 5 patients showed cystic changes in the right hypogastrium inclu-ding irregular shape with lobulation in 4 and tubular shape in 1.The maximum traverse diameters ranged from 27 mm to 146 mm. Unclear boundary was showed if the lesion invaded the adjacent gut.The cystic wall was asymmetry and thickened with little nodes and nipple in the inner wall.The thick septa were seen in 3 tumors.Much mucus was seen in tumors with uneven density and average CT value of 1 9.3 HU-34.6 HU.On enhanced CT,obvious enhancement of the cystic wall and septa in 4 and moderate enhance-ment in 1 was showed.The adjacent fatty gap of 3 tumors were clear,however it was vague when accompanied with acute inflamma-tion.Small calcification in 3 and enlarged lymphatic nodes in 3 were also found.Conclusion CT shows some characteristics of mu-cinous cystadenocarcinoma of appendix and plays an important role in the diagnosis of the disease.

Pulmonary Mucinous Cystadenocarcinoma: Report a Case and Review of CT Findings

A pulmonary mucinous cystadenocarcinoma is an extremely rare tumor that is considered to be a cystic variant of mucin-producing lung adenocarcinoma. We present a case of pulmonary mucinous cystadenocarcinoma in a 54-year-old woman. Chest CT scans showed a 4.3-cm-sized, lobulated, well-defined, and homogeneous mass in the right middle lobe with peripheral stippled calcifications that demonstrated low-attenuation with no enhancement after contrast administration; 18F-fluorodeoxyglucose (FDG) PET/CT demonstrated mild heterogeneous FDG uptake. The mass was diagnosed as adenocarcinoma with mucin production by transbronchial lung biopsy. Right middle lobectomy was performed, and the pathologic examination disclosed a pulmonary mucinous cystadenocarcinoma.

A Case of Sarcomatoid Carcinoma Arising from Mucinous Cystadenocarcinoma of Appendix

Sarcomatoid carcinoma or carcinosarcoma is a very rare biphasic tumor characterized by a combination of malignant epithelial and mesenchymal cells. The pathogenesis of sarcomatoid carcinoma is not fully elucidated and the guideline of treatment has not been established yet. Although the upper aerodigestive tract, lung and female urogenital system are known to be the most frequently affected, this tumor also can occur in various sites, including the digestive tract. Since sarcomatoid carcinoma in colon was firstly reported in 1986, 24 cases have been reported to date. We report a rare case with sarcomatoid carcinoma of appendix. Interesting histologic feature of our case was the presence of mucinous cystadenocarcinoma with morphological "transition" between carcinomatous and sarcomatous tissue. To our knowledge, this is the first case of sarcomatoid carcinoma arising from mucinous cystadenocarcinoma of the appendix.

MR Imaging of Primary Retroperitoneal Mucinous Cystadenocarcinoma in Pregnant Woman

Primary retroperitoneal mucinous cystadenocarcinoma is a very rare malignancy. To date, 51 cases have been reported, including 3 in pregnant women. Herein, we report magnetic resonance findings of a 31-year-old Korean woman (15 weeks and 3 days pregnant) with primary retroperitoneal mucinous cystadenocarcinoma. On abdominal magnetic resonance imaging (MRI), a mass was identified in the retroperitoneal area with a nodular lesion showing heterogeneous signal intensity and focal wall thickening on T1- and T2-weighted images. Exploratory laparotomy and tumor excision were performed. Histological examination revealed primary retroperitoneal mucinous cystadenocarcinoma. The patient subsequently underwent total hysterectomy, bilateral salpingo-oophorectomy, and omentectomy for metastatic mucinous cystadenocarcinoma of both ovaries 15 months after her initial surgery.

Primary Mucinous Cystadenocarcinoma of the Breast: Cytologic Finding and Expression of MUC5 Are Different from Mucinous Carcinoma

Mucinous cystadenocarcinoma (MCA) in the breast is a rare neoplasm. There have been 13 cases of primary breast MCA reported. The MCA presents as a large, partially cystic mass in postmenopausal woman with a good prognosis. The microscopic findings resemble those of ovarian, pancreatic, or appendiceal MCA. The aspiration findings showed mucin-containing cell clusters in the background of mucin and necrotic material. The cell clusters had intracytoplasmic mucin displacing atypical nuclei to the periphery. Histologically, the tumor revealed an abundant mucin pool with small floating clusters of mucin-containing tumor cells. There were also small cysts lined by a single layer of tall columnar mucinous cells, resembling those of the uterine endocervix. The cancer cells were positive for mucin (MUC) 5 and negative for MUC2 and MUC6. This mucin profile is different from ordinary mucinous carcinoma and may be a unique characteristic of breast MCA.

A Case of Skin Metastasis of Ovarian Cancer (Mucinous Cystadenocarcinoma) / 대한피부과학회지

Metastasis to the skin from internal malignant neoplasms is an uncommon event. Although carcinoma of the ovary is the second most common malignancy in women, cutaneous involvement originating from ovarian cancer is particularly unusual. A 30-year-old woman had been treated with chemotherapy for ovarian cancer 4 years previously. She showed an erythematous indurated subcutaneous nodule on her left shoulder and scalp. Histopathologic findings showed multiple cribriform pattern cellular masses and some cords or tubular structures floating on a mucinous pool, which were similar to primary mucinous cystadenocarcinoma. We report this case of cutaneous metastasis from ovarian cancer.

A case of mucinous adenocarcinoma arising from retroperitoneal teratoma treated with chemoradiation / 부인종양

Retroperitoneal teratoma with malignant transformation is a rare condition in adults. Its most common malignant transformation is into a squamous cell carcinoma, but rarely into a mucinous adenocarcinoma. Postoperative treatment of mucinous adenocarcinoma arising from teratomas has not been established due to its rare incidence. Here we present a case of retroperitoneal mucinous adenocarcinoma arising from a teratoma in the presacral area. Operative and postoperative managements are described with a brief review of the literatures.

A case of small cell carcinoma of pulmonary type of ovary associated with huge mucinous cystadenocarcinoma / 대한산부인과학회지

Small cell carcinoma of ovary is extremely rare. This neoplasms compose predominantly or exclusively of small round cells with scant cytoplasm. There are two types of primary small cell carcinoma of ovary described in the literature : the hypercalcemic type and the pulmonary type. The first 11 patients with a primary ovarian small cell carcinoma of the pulmonary type were described by Eichorn et al. in 1992. The prognosis of this tumor is very poor and only limited data on the treatment of this tumor type are available. In the present case report, we describe a patient with the pulmonary type of small cell carcinoma associated with huge mucinous cystadenocarcinoma with a brief review of the concerned literatures.

An ovarian mucinous cystadenocarcinoma arising from mature cystic teratoma with para-aortic lymph node metastasis: a case report / 부인종양

Malignant transformation of a mature cystic teratoma (MCT) is an uncommon complication. The most common form of malignant transformation of a MCT is squamous cell carcinoma, representing 75% of malignant transformations. The frequency of malignant transformation of MCT to adenocarcinoma is just 6.8%. To the best of our knowledge, no case of para-aortic lymph node metastasis in mucinous adenocarcinoma arising from MCT has been reported before. The prognosis of malignant transformation of the MCT is very poor. Here, we report an unusual case of a 41-year-old woman with mucinous adenocarcinoma arising from MCT with para-aortic lymph node metastasis.

An ovarian mucinous cystadenocarcinoma arising from mature cystic teratoma with para-aortic lymph node metastasis: a case report / 부인종양

Malignant transformation of a mature cystic teratoma (MCT) is an uncommon complication. The most common form of malignant transformation of a MCT is squamous cell carcinoma, representing 75% of malignant transformations. The frequency of malignant transformation of MCT to adenocarcinoma is just 6.8%. To the best of our knowledge, no case of para-aortic lymph node metastasis in mucinous adenocarcinoma arising from MCT has been reported before. The prognosis of malignant transformation of the MCT is very poor. Here, we report an unusual case of a 41-year-old woman with mucinous adenocarcinoma arising from MCT with para-aortic lymph node metastasis.