Search

Adenomatoid tumors (AT) arising in the female genital tract are usually incidental findings occurring most often in the fallopian tube and uterine serosa and rarely in the myometrium. In the myometrium, they appear grossly as deep seated, small, firm, ill circumscribed nodules mimicking leiomyoma. Histologically they show a glandular and invasive pattern making well-differentiated/low-grade endometrioid adenocarcinoma a major differential diagnosis. However, this differential is rarely encountered in practice because myometrial AT is usually seen on the hysterectomy specimen, because of their anatomic position in the deep myometrium, and only rarely in endometrial curettings. Our case is the first to report an AT, which presented as a polyp with associated fibroid on hysterescopic examination. Microscopically, the endometrial curetting and myomectomy showed irregular glands and cystic structures with occasional cytokeratin positive single signet-ring like cells invading into the myometrium, features consistent with low-grade endometrioid adenocarcinoma. On hysterectomy specimen, there was an ill-defined 5 cm mass in the myometrium with protrusion into the endometrium. The morphology was similar to that seen in the endometrial curetting. A larger panel of immunostains was done and the neoplastic cells were positive for AE1/3, CK7, CAM5.2, calretinin, and D2-40 and negative for CD34. A diagnosis of AT was rendered and no further treatment was required. Although AT is rarely seen in endometrial curetting, they should be in the differential diagnosis of glandular lesions to avoid pitfalls and unnecessary management especially in young patients desiring fertility.

This is a case of a 62-year-old woman with a remote history of acinic cell carcinoma of the parotid gland, who presented with a palpable vaginal mass, anterior vaginal wall prolapse, and stress urinary incontinence. A 2 cm firm mobile mass on the anterior vaginal wall was found on clinical examination. A computed tomographic scan revealed a mass between the vaginal vault and bladder that was eventually surgically excised. The histology, supported by the immunohistochemistry, revealed metastatic acinic cell carcinoma to the vagina after 37 years of her initial diagnosis. This is the first reported case in the literature to occur in the vagina.

We have previously shown that HOMER2 (Homer scaffolding protein 2), a protein coding gene, was highly expressed in low grade (LG) endometrioid adenocarcinoma (EAC) of the uterus. The role of HOMER2 in endometrial cancer (EC) is widely unknown; therefore, the aim of this study was to determine the expression and the predictive value of HOMER2 protein expression in series of patients with EC. HOMER2 protein expression was detected on paraffin-embedded tissues from 336 cases using immunohistochemistry (IHC). Tumours were categorised in two groups; group 1 (EAC, FIGO grade 1 and 2; n = 191) and group 2 (all other subtypes including grade 3 EAC; n = 145). Statistical analysis was performed to evaluate associations between HOMER2 protein expression and pathological parameters (histological type, grade, stage, lymphovascular invasion, myometrial depth of invasion) and patient outcome [progression-free survival (PFS) and cancer-specific survival (CSS)]. HOMER2 was significantly overexpressed in group 1 compared to group 2 cancers (67% versus 30%; p < 0.001) and with low tumour grade (p < 0.001). In group 1, HOMER2 overexpression was an independent prognostic factor for improved CSS (adjusted-hazard ratio 0.28; 95% confidence interval 0.08-0.96; p = 0.042). HOMER2 expression was not associated with survival in group 2 (p > 0.05). This is the first study of HOMER2 protein expression in EC. We speculate that HOMER2 may be involved in tumourigenesis of endometrioid uterine tumours and suggest that HOMER2 should be studied further for potential clinical and therapeutic applications., (Copyright © 2018 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.)

The purpose of this study is to assess the reproducibility among gynaecological pathologists in their diagnosis of mucinous alterations in endometrial sampling specimens. Twenty-six cases were independently reviewed by four experienced gynaecological pathologists from four academic medical centres. Pathologists were asked to classify each case into one of four groups, including three World Health Organization (WHO)-recognised categories: (1) mucinous metaplasia; (2) atypical mucinous glandular proliferation; (3) carcinoma; and (4) 'other' (absence of a true mucinous alteration and/or an alteration of non-endometrial origin). The overall reproducibility was 'fair' (κ = 0.39). In an analytical scenario that established three clinically significant groups ('benign/non-neoplastic', 'atypical', and 'carcinoma') by redistributing all group 4 responses, the resultant kappa improved to 0.51 (moderate reproducibility). In another analysis with only two categories-'benign/non-neoplastic' versus 'atypical/carcinoma'-reproducibility was similarly moderate (κ = 0.46). However, with one exception, all cases that were ultimately diagnosed as carcinoma in a follow-up hysterectomy specimen, were classified as atypical or carcinoma in the preceding sampling. For 11 cases that were classified as either 'carcinoma' or 'atypical' by all observers, there was moderate reproducibility (κ = 0.53) in making that distinction, and none of a wide array of morphological features were found to significantly distinguish between these two categories. For five cases that all observers classified as either mucinous metaplasia or benign endocervix, reproducibility was substantial (κ = 0.67). In summary, gynaecological pathologists show moderate reproducibility in categorising mucinous alterations in endometrial sampling specimens as benign, atypical, or carcinomatous. They accurately classify as at least 'atypical' those cases that are ultimately diagnosed as carcinoma in the subsequent resection. Our findings suggest that there are indeed some mucinous alterations which have features that do not allow for reproducible assignment by pathologists into the WHO-recognised categories. In this subset of cases, there may be a need for better-defined diagnostic criteria and/or extra-morphological diagnostic tools., (Copyright © 2018 Royal College of Pathologists of Australasia. Published by Elsevier B.V. All rights reserved.)

A 56-year-old G3P3 postmenopausal woman presented with a 5 month history of abnormal uterine bleeding and pelvic pain. A computed tomographic scan revealed a 5 cm right adnexal cystic mass in addition to a thickened, heterogenous endometrium and leiomyomatous uterus. A total laparascopic hysterectomy and bilateral salpingo-oophorectomy with omental and peritoneal biopsy were performed. Gross examination revealed a 12 week size uterus with small fibroids, normal bilateral atrophic ovaries, and a right paratubal cyst. A 4 cm vegetating mass was found in the right side of the uterine wall. Microscopically, the uterine mass was diagnosed as an endometrioid adenocarcinoma (EAC) FIGO 1 with 70% of myometrial invasion. The remaining endometrium showed a complex atypical hyperplasia. In addition, a 5 cm paratubal cystic mass was found that was separate from the uterus and the right adnexa. The cyst content was a chocolate brown fluid and the cyst wall was smooth with a single solid mass of 2 cm in size. The diagnosis of EAC, FIGO 1 was given. The remaining cyst lining showed endometriotic cyst and foci of endometriosis in the cyst wall. There was no lymphovascular invasion. The entire fallopian tube and ovaries were submitted and they were free of tumor. The patient was diagnosed with primary EAC of the paratubal cyst in addition to EAC of the uterine corpus (pT1b). A close follow-up was recommended. Because of our limited knowledge of carcinomas arising in the paratubal cyst, we will review the literature and discuss their clinical aspects, management, and behavior.

We present a case of a 45-year-old woman with a long-standing history of persistent cervical dysplasia that resulted in a hysterectomy. Subsequent vaginal smears revealed high-grade vaginal intraepithelial neoplasia (VAIN III) on Pap smear with positive human papilloma virus (HPV) testing. Over the course of 2 years, the patient underwent 2 CO(2) laser vaporization procedures of the upper vagina and intermittent 5-fluorouracil therapy. A biopsy performed at the time of the second laser procedure revealed endocervical-type well-differentiated adenocarcinoma, associated with VAIN III. HPV in situ hybridization for HPV types 16 and 18 was positive in both the glandular and squamous mucosa. The patient has no known history of intrauterine diethylstilbestrol exposure or mullerian developmental abnormalities. Subsequently, the patient underwent a radical upper vaginetcomy with bilateral pelvic lymph nodes dissection and bilateral salpingo-oophorectomy. The vaginectomy specimen showed residual adenocarcinoma associated with VAIN-III and extensive vaginal adenosis with free resection margins. This is the second reported case in the literature of adenocarcinoma arising in vaginal adenosis after 5-fluorouracil. Herein, we highlight these important findings and shed some light on the pathogenesis of vaginal adenosis and the subsequent development of vaginal adenocarcinoma.

Epithelioid sarcoma of the vulva is an extremely rare neoplasm with aggressive behavior and poor outcome. Herein, we report a case of vulvar epithelioid sarcoma presenting in a 17-year-old very early in her pregnancy. The patient presented with an asymptomatic nodule of the right labia majora of 1-year duration. Computerized tomographic scans showed enlarged inguinal lymph nodes and numerous lung nodules. Positron emission tomography was performed and revealed no suspicious lesions for metastatic disease. The patient underwent local excision of her vulvar lesion. On the basis of morphology and extensive immunohistochemistry, the lesion was classified as epithelioid sarcoma. The patient was referred to radiation therapy and upon evaluation, she was found to be in her sixth week of gestation. The patient continued with her pregnancy and underwent a lymph node dissection, which was positive. We review the literature of only the well-documented cases extensively studied by immunohistochemical analysis. We summarize the clinical presentation, clinical impression, treatment modalities, and outcomes of these cases. On account of the rarity of this disease in the vulva, there is no clear consensus on treatment modalities, but it seems that early aggressive surgical resection is the treatment of choice, with the role of adjuvant therapy to be determined.

Distinguishing primary from metastatic disease is vital for patient prognosis and care. This becomes a difficult task for both clinicians and pathologists when the clinical data, morphology, and immunohistochemistry (IHC) are not congruent. Array-based comparative genomic hybridization (aCGH) is a fairly recent technique that enables the analysis of whole tumor genome for discreet genetic abnormalities. As it is applicable to paraffin-embedded tissue, it could be suited for surgical pathology settings. We report a case of a 71-year-old woman diagnosed with uterine carcinosarcoma associated with high-grade neuroendocrine carcinoma (NEC). Six months later, the patient presented with pulmonary nodules. A wedge resection of one the nodules was performed and the histology and IHC were consistent with NEC. However, the diagnostic dilemma was whether the pulmonary lesions were primary of the lung or metastatic from the NEC component of the endometrium. Clinical, morphologic, and IHC analysis were not helpful to answer the above question. However, by revealing different genetic profiles of each of the 2 tumors, aCGH was suggestive of the existence of 2 independent primary tumors one endometrial and second pulmonary. In conclusion, aCGH is a powerful tool that is capable of revealing tumor gene fingerprinting and it can be used in routine surgical pathology especially in those cases where the other conventional techniques are not helpful.

We report 2 cases of desmoplastic small round cell tumor (DSRCT) involving the ovaries in young women. The first patient presented with symptoms of acute appendicitis and the second patient presented with a mass in the lower abdomen and slightly elevated CA-125 level. In both patients, the tumor was widely metastatic at presentation. The ovarian involvement was unilateral in the first patient and bilateral in the second with tumor size ranging from 9 to 11 cm. Morphology, immunohistochemistry, and molecular cytogenetics were consistent with DSRCT. Despite tumor debulking and multiple chemotherapy regimens, the first patient died at 20 months after initial diagnosis and the second is still undergoing chemotherapy at 7 months after initial presentation. To gain additional insight on DSRCT with ovarian involvement, the literature was reviewed and summarized.