Ovarian Clear Cell Tumors Associated With Seromucinous Borderline Tumor: A Case Series.

Ovarian seromucinous borderline tumors (SMBT) and clear cell tumors are both closely associated with endometriosis and share, in a proportion of cases, a molecular pathway involving ARID1A mutations, but they have been rarely described in association. We report a case series of 4 clear cell tumors (3 carcinomas, 1 borderline adenofibroma) coexisting in the same ovary with SMBT. In all cases, the SMBT was the predominant component and we highlight that adequate sampling of these tumors is important to detect small clear cell carcinomas, thus potentially altering the treatment and prognosis.

Magnetic Resonance Imaging Findings of Biliary Adenofibroma.

Biliary adenofibroma is a rare tumor with a bile duct origin characterized by a complex tubulocystic non-mucin secreting biliary epithelium with abundant fibrous stroma. The MRI features of biliary adenofibroma are not well established. The authors encountered two patients with biliary adenofibroma and reviewed the literature focusing on the MRI findings. A well-circumscribed multicystic tumor with septal enhancement and no intrahepatic bile duct communication may be the characteristic MRI findings of biliary adenofibroma.

Malignant Transformation Arising Within Unusual and Rare Hepatic Lesions: Fibropolycystic Disease Form of Ductal Plate Malformation and Biliary Adenofibroma.

Cholangiocarcinoma is the second most common hepatobiliary cancer following hepatocellular carcinoma, and 20% to 25% are intrahepatic. We describe 2 cases of intrahepatic cholangiocarcinoma arising within unusual and rare hepatic lesions, fibropolycystic liver disease form of ductal plate malformation and biliary adenofibroma, whose association with malignancy is rarely reported in the literature.

Is target oriented surgery sufficient with borderline ovarian tumors? - Role of accompanying pathologies.

BACKGROUND: There are limited data in the literature related to concomitant genital or extra-genital organ pathologies in patients with borderline ovarian tumors (BOTs). The aim of this study was to evaluate our experience with 183 patients to draw attention to the accompanying organ pathologies with BOTs. MATERIALS AND METHODS: One hundred eighty-three patients with BOTs, diagnosed and/or treated in our center between January of 2000 and March of 2013 were evaluated retrospectively. Data related to age, tumor histology, lesion side, disease stage, accompanying incidental ipsilateral and/or contralateral ovarian pathologies, treatment approaches, and follow-up periods were investigated. Incidental gynecologic and non-gynecologic concomitant organ pathologies were also recorded. RESULTS: The mean age at diagnosis was 40.6 years (range: 17-78). Ninety- five patients (51%) were ≤40 years. A hundred and forty-seven patients (80%) were at stage IA of the disease. The most common type of BOT was serous in histology. Non-invasive tumor implants were diagnosed in 4% and uterine involvement was found 2% among patients who underwent hysterectomies. There were 12 patients with positive peritoneal washings. Only 17 and 84 patients respectively had concomitant ipsilateral and concomitant contralateral incidental ovarian pathologies. The most common type of uterine, appendicular and omental pathologies were chronic cervicitis, lymphoid hyperplasia and chronic inflammatory reaction. CONCLUSIONS: According to our findings most of accompanying pathologies for BOT are benign in nature. Nevertheless, there were additional malignant diseases necessitating further therapy. We emphasize the importance of the evaluation of all abdominal organs during surgery.

Tumor mixto epitelial y estromal del riñón con diferenciación endometrioide asociado a un tumor sólido seudopapilar de páncreas / Mixed epithelial and stromal tumor of the kidney with endometrioid differentiation associated with pancreatic solid pseudopapillary tumor

El tumor mixto epitelial y estromal del riñón (MESTK) es una neoplasia quística benigna. El tumor sólido seudopapilar pancreático (SPT) es un carcinoma quístico de bajo potencial maligno. Este artículo describe un MESTK sincrónico con un SPT en una mujer con un cuerpo lúteo quístico ovárico. Las glándulas mostraban diferenciación endometrioide y el estroma fusocelular creaba complejas estructuras foliáceas similares a un adenofibroma mulleriano. El SPT estaba formado por células poligonales con patrón seudopapilar y un área quística. La expresión de receptores de estrógenos y progesterona en las células estromales del MESTK y de receptores de progesterona en el SPT es clave para su diagnóstico, confirmando una dependencia hormonal en la patogénesis. Dado que nuestro MESTK es polipoide y tiene diferenciación endometrioide, discutimos el diagnóstico diferencial con la endometriosis polipoide(AU)

Mixed epithelial and stromal tumor of the kidney (MESTK) is a benign cystic neoplasm. Pancreatic solid-pseudopapillary tumor (SPT) is a typically cystic carcinoma of low malignant potential. We describe a MESTK synchronous with a SPT in a female with an ovarian cystic corpus luteum. Glands showed endometrioid differentiation and the spindle cell stroma created complex leaflet-like structures similar to Müllerian adenofibroma. The SPT consisted of polygonal cells with a pseudopapillary pattern and a cystic area. Expression of estrogen and progesterone receptors in MESTK stromal cells and progesterone receptors in SPT provided a diagnostic clue, confirming the hormonal dependence in pathogenesis. Given that our MESTK is a polypoid tumor with endometrioid differentiation, the differential diagnosis with polypoid endometriosis is considered(AU)

Borderline clear cell adenofibroma of the ovary associated with ovarian endometriosis: a case report.

Clear cell tumours of the ovary are relatively uncommon. Most of them are clear cell carcinomas. Benign and borderline clear cell tumours are extremely rare and almost always fibromatous. We report a case of a 34-year-old woman. Ultrasound and computed tomography showed a right ovarian mass 8 cm in diameter. The patient underwent right salpingo-oophorectomy. Microscopic examination revealed glands and cysts different in size and shape within an abundant stromal component without evidence of stromal invasion. Many cysts and glands were lined by a single layer of flattened, cuboidal or hobnail cells with mild to moderate cytologic atypia and prominent nucleoli. Psammomatous calcifications were occasionally indentified. Features of endometriosis were also present adjacent to the tumour. Lesional cells were positive for Ker 7 and CA125. Staining for p53 was focally positive. Based on the above characteristic morphologic and immunohistochemical findings a diagnosis of borderline clear cell adenofibroma was made. The patient was free of recurrence four years after surgery.

Adenofibroma and ectopic pregnancy of left fallopian tube: a rare coexistence.

A rare coexistence of an adenofibroma and ectopic pregnancy in the left fallopian tube is reported. Adenofibroma is a rare benign neoplasm of the fallopian tube, and the fallopian tube is the most common site of ectopic pregnancies. However, coexistence of such rare tumor and ectopic pregnancy is extremely rare. Review of literature has not revealed another case of such coexistence in English literature. In the present case, the adenofibroma was found at the fimbrial end. Placental tissue, consisting of chorionic villi and trophoblastic cells was present in the ampulla of fallopian tube.

An allelotype analysis indicating the presence of two distinct ovarian clear-cell carcinogenic pathways: endometriosis-associated pathway vs. clear-cell adenofibroma-associated pathway.

Patterns of allele loss (loss of heterozygosity (LOH)) were studied to identify the genetic backgrounds underlying the two putative carcinogenic pathways of ovarian clear-cell adenocarcinoma: carcinomas thought to arise in endometriosis (endometriosis-associated carcinomas, 20 cases) and carcinomas thought to be derived from clear-cell adenofibroma ((CCAF)-associated carcinomas, 14 cases). Each tumor was assessed for LOH at 24 polymorphic loci located on 12 chromosomal arms: 1p, 3p, 5q, 8p, 9p, 10q, 11q, 13q, 17p, 17q, 19p, and 22q. For all informative loci, the frequency of LOH was not statistically different between the two carcinoma groups: 38% (66/172 loci) in the endometriosis-associated carcinomas and 35% (40/113 loci) in the CCAF-associated carcinomas. In the endometriosis-associated carcinomas, LOH was detected at high frequencies (>50%) at 3p, 5q, and 11q and at low frequencies (<20%) at 8p, 13q, and 17p. In the CCAF-associated carcinomas, LOH was detected at high frequencies at 1p, 10q, and 13q and at low frequencies at 3p, 9p, 11q, and 17q. The frequencies of LOH at chromosomes 3p, 5q, and 11q were significantly higher in the endometriosis-associated carcinomas than in the CCAF-associated carcinomas (P = 0.026, 0.007, and 0.011, respectively). Immunohistochemical analysis demonstrated a close association between the allelic status of the 3p25-26 locus and levels of von Hippel-Lindau (VHL) protein expression (P = 0.0026). These data further support the presence of two distinct carcinogenic pathways to ovarian clear-cell adenocarcinoma; the allelic status of the 3p, 5q, and 11q loci may provide a means to identify the precursor lesions of these carcinomas.

Adenofibroma of the testis.

Adenofibromas of the testicular parenchyma are exceptional with only one case published in the literature. Whereas adenofibromas of mullerian derivatives are well described, testicular tumours resembling surface ovarian epithelium are rare. We describe a case of adenofibroma discovered incidentally while investigating for a hydrocoele in a 57-year-old patient. Histological and immunohistochemical evaluation confirmed the lesion as being a serous adenofibroma with immunohistochemical properties analogous to its ovarian counterpart. These lesions are to be considered in the differential diagnosis of unusual cystic lesions of the testis, in order not to confuse it with more sinister pathology.

Extensive pelvic calcification in association with ovarian serous cystadenofibroma.

We describe the first reported case of extensive calcification seen in association with serous cystadenofibroma of the ovary, illustrating that calcification in the female genital tract may be extensive in nature, presenting in a fashion that is not entirely typical of dystrophic or metastatic calcification. This case demonstrates that extensive calcification within the pelvis should be interpreted with caution, as it may not represent disseminated malignancy.

Oclusión colónica por tumor ovárico / Colonic occlusion due to ovarian tumor

La obstrucción intestinal constituye una de las causas más frecuentes de abdomen agudo, que suele ser mortal en ancianos por concomitar con enfermedades neoplásicas malignas del colon; sin embargo, es raro encontrar tumores benignos del aparato ginecológico en mujeres posmenopáusicas, que obstruyan mecánica y completamente el colon por compresión extrínseca. En este artículo se describe el caso clínico inusual de una paciente de 80 años de edad, operada a causa de una oclusión del intestino grueso por un cistoadenofibroma de ovario

The intestinal obstruction constitutes one of the most frequent causes of acute abdomen, which is usually fatal in elderly as it is accompanied by malignant neoplasic diseases of the colon. However, it is uncommon to find benign tumors of the gynecologic apparatus that obstruct the colon mechanically and completely by extrinsic compression in postmenopausic women. In this article the unusual clinical case of a 80 year-old patient is described, who was operated due to an occlusion of the large intestine by an ovarian cystoadenofibroma

A menopausal woman with mullerian agenesis, a leiomyoma, an inguinal hernia, and cystadenofibromas.

BACKGROUND: Müllerian agenesis, the second most common cause of primary amenorrhea, affects 1 in 4,000 to 10,000 women. Although the majority of these women have complete absence of the uterus, a small percentage can have a rudimentary uterus. CASE: A menopausal nulligravida with known müllerian agenesis developed a large pelvic mass and an inguinal hernia. The patient underwent an exploratory laparotomy, which revealed a large leiomyoma growing from a small rudimentary uterus, an indirect inguinal hernia, and bilateral benign serous cystadenofibroma. CONCLUSION: Women with müllerian agenesis and rudimentary uteri can develop gynecologic pathology similar to women with normal genital tracts.

[A young patient with endometrioid adenocarcinoma who suffered Trousseau's syndrome associated with vasculitis].

A 27-year-old woman was admitted to our hospital because of headache, fever and right neck pain. Neurological examination revealed mild meningeal signs, and hyper-reflexia in all extremities. In the laboratory tests, white-cell count was 13,000/mm3, rheumatoid factor(RF) and C-reactive protein(CRP) were positive. The cerebro-spinal fluid showed pleocytosis (56/mm3, neutorophils and lymphocytes were 26 and 28, respectively). Thus, she was diagnosed as aseptic meningitis. A few days later, she had weakness and dysesthesia of the right face and the left extremities. Pulse therapy with intravenous methylprednisolone was started. A magnetic resonance imaging (MRI) of the brain showed a hemorrhagic infarction in the right parietal lobe. In hemostatic markers, thrombin-antithrombin III complex(TAT; 106 ng/dl), D-dimer 1234 ng/dl, prothrombin fragment 1 + 2(F1 + 2; 2.36 nmol/L), beta-thromboglobulin (beta TG; 4,300 ng/dl) and platelet factor 4 (PF-4; 1,770 ng/dl) were extremely elevated. On duplex ultrasonography, a low echo lucent plaque was observed at the right internal carotid artery and the mean blood flow velocity in the right carotid artery was decreased. She was placed on oral prednisolone and warfarin for suspected stroke due to hypercoagulability associated with vasculitis. Afterwards, she discharged from our hospital. Two months later, she was readmitted to our hospital because of irregular menses and vaginal bleeding. Endometrial uterus biopsy was conducted, which revealed a grade I endometrioid adenocarcinoma. She was under total uterectomy without tumor recurrence. After the radical operation, white-cell count, RF, CRP, TAT, D-dimer, F1 + 2, and beta TG were normalized, and the mean flow velocity of the right common carotid artery was increased. Thereafter, she did not experience stroke recurrence. Therefore, we speculated that she had stroke due to hypercoagulability in association with malignancy, that is Trousseau's syndrome. We also assumed that aseptic meningitis, brainstem encephalitis associated with vasculitis in this patient are other clinical variants of paraneoplastic syndrome through immunological mechanisms associated with malignancy. We emphasize that patients with Trousseau's syndrome can be associated with other paraneoplastic manifestations such as vasculitis as seen in this patient.

Uterine cervical adenofibroma associated with Turner's syndrome in a young woman.

A 32-year-old woman with Turner's syndrome complained of abnormal genital bleeding, which was diagnosed as a delivered myoma. Vaginal myomectomy was performed, and the tumor was histopathologically diagnosed as an adenofibroma of the uterine corpus. As these are extremely rare, we report this case with a review of some of the literature.

The association of benign and malignant ovarian adenofibromas with breast cancer and thyroid disorders.

An unexpected association with breast cancer and thyroid disorders was found during a review of 91 cases of benign and malignant ovarian adenofibromas. Sixty-three tumors were benign, 11 had areas of borderline neoplasms, and 17 had a component of carcinoma. Such tumors were divided into glandular/cystic (61 cases) and papillary (30 cases) according to their gross and microscopic appearance. Among the 61 patients with glandular/cystic adenofibromas, 13 (21%) had breast cancer and 19 (31%) also had thyroid disorders. Among the 30 patients with papillary adenofibromas there were no cases of breast cancer and only 2 patients had thyroid disorders. The average age of the patients with ovarian adenofibroma and breast cancer or thyroid disorders was higher (66 years) than that of patients without breast cancer or thyroid disorders (55 years). More patients with breast cancer and thyroid disorders had bilateral adenofibromas than patients without breast cancer or thyroid disorders. We also reviewed the medical records of 100 patients with ovarian cancer without adenofibroma component, 100 patients with breast cancer, and 100 patients with ovarian and breast cancer. Six percent of patients with ovarian cancer had breast cancer and 16% of each one of these groups had thyroid disorders. This unexpected association found between glandular/cystic adenofibromas, breast cancer, and thyroid disorders might be explained by defects common to these organs. Disorders of some of these organs have been linked by common genetic changes and it is known that these organs are under the influence of similar hormones. Mutations of PTEN have been found in breast and thyroid cancer. The thyroid and ovaries are controlled by glycoprotein hormones of the pituitary gland, which have common alpha subunits.

Metrorragias seniles. Estudio clínico, histeroscópico e histológico de 184 casos / Senile metrorrhagias. A clinical study, hysteroscopy and histopathology of 184 cases

Presentamos 184 casos de metrorragias seniles, en mujeres de 70 o más años de edad con nuestra experiencia durante 10 años en histeroscopia diagnóstica y quirúrgica, haciendo un estudio clínico, histeroscópico e histológico. Las metrorragias seniles representan el 16,31 por ciento de las metrorragias posmenopáusicas. El 46,73 por ciento se dan en la franja de edad de 71-75 años. El cáncer de endometrio ocurre en el 18,57 por ciento de los casos. En el 2,71 por ciento la histeroscopia no es decisoria por diversos motivos. En un 50,58 por ciento se resuelve la patología por histeroscopia quirúrgica, para pólipos, miomas e hiperplasias; la histerectomía se reserva para casos con atipias o no decisorios (5,88 por ciento). Globalmente, se practica en un 42,35 por ciento por los casos de cáncer de endometrio o cérvix. Las mujeres ancianas necesitan una atención ginecológica adecuada para el diagnóstico y tratamiento de la metrorragia senil, por la frecuencia de cáncer endometrial a estas edades. (AU)

Neoplastic and pre-neoplastic changes in gastrointestinal endometriosis: a study of 17 cases.

The clinicopathologic features of neoplasms arising in gastrointestinal endometriosis have not been well characterized. In this series, we report 17 cases of gastrointestinal endometriosis complicated by neoplasms (14 cases) or precancerous changes (three cases). Four patients, one of whom also had hypermenorrhea, presented with chronic abdominal pain and five had obstructive symptoms; one of these also had rectal bleeding. One patient presented with an acute abdomen and fecal peritonitis, one had vaginal bleeding, and one had a progressive change in bowel habits. Nine patients had a long history of endometriosis, 11 patients had had hysterectomies, and eight of these had also received unopposed estrogen therapy. The lesions involved the rectum (6), sigmoid (6), colon, unspecified (2), and small intestine (3), and comprised 8 endometrioid adenocarcinomas (EA), 4 mullerian adenosarcomas (MAS), 1 endometrioid stromal sarcoma (ESS), 1 endometrioid adenofibroma of borderline malignancy (EBA) with carcinoma in situ, 2 atypical hyperplasias (AH), and one endometrioid adenocarcinoma in situ (ACIS). The tumors ranged in size from 2 to 15 cm and all involved the serosa and muscularis propria. Two tumors extended into the mucosa, with mucosal ulceration in one. Follow-up was available in 11 cases. One patient with EA was dead of disease at 1 year, one had two recurrences at 1 and 2 years, and three were alive with no evidence of disease (ANED) at 9 months to 13 years (mean, 68 mos). The patient with the EBA was ANED at 3 months. Two patients with MAS were ANED at 2 and 3 years. The patient with ESS had a recurrence at 3 years and was ANED 6 years after her original diagnosis. One woman with AH was ANED at 60 months and the patient with ACIS was ANED at 16 months. One of the carcinomas was originally misdiagnosed as a primary intestinal adenocarcinoma. The pathologist should be aware of the possibility of a tumor of genital tract type when evaluating intestinal neoplasms in females, particularly if they have a history of endometriosis and have received unopposed estrogen therapy.