Wolffian adnexal tumor, so-called female adnexal tumor of probable Wolffian origin (FATWO): immunohistochemical evidence in support of a Wolffian origin.

Wolffian adnexal tumor (WAT) is a rare neoplasm believed to originate from wolffian remnants on the basis of its location in areas where these remnants are abundant. To study its histogenesis, the immunoprofile of 25 WATs was compared with that of 10 cervical and vaginal mesonephric remnants and 12 rete ovarii. WATs were unilaterally located in the broad ligament (n = 10), mesosalpinx (n = 9), ovarian hilus (n = 5), and pelvis, not otherwise specified (n = 1). They showed varying morphologies with solid (spindle cells), tubular (lined by columnar cells), retiform and multicystic (spaces lined by cuboidal and attenuated cells) patterns. WATs were immunoreactive for pan-cytokeratin (AE1/3, CK1) (100%), CAM 5.2 (100%), cytokeratin 7 (CK7) (88%, focal staining), keratin 903 (17%), epithelial membrane antigen (EMA) (12%), estrogen receptor (28%), progesterone receptor (24%), androgen receptor (78%), inhibin (68%), calretinin (91%), and vimentin (100%). No immunostaining was detected with monoclonal carcinoembryonic antigen and cytokeratin 20. The pattern of staining was nearly identical to that of the rete ovarii and differed somewhat from mesonephric remnants, which were diffusely immunoreactive for CK7, immunopositive for EMA (apical staining), and nonreactive for inhibin. Our findings provide immunohistochemical support for the derivation of WATs from wolffian remnants, in particular from the rete ovarii. Because of immunoreactivity for inhibin and calretinin in a significant number of WATs, our results further show that these immunostains alone do not allow absolute distinction of WATs from sex cord-stromal tumors and adenomatoid tumors, respectively, with which they may be confused.     

Metastatic female adnexal tumor of probable Wolffian origin: a case report and review of the literature

Female adnexal tumor of probable wolffian origin is a rare neoplasm that can present diagnostic difficulties. We report herein a case of a 60-year-old woman with female adnexal tumor of probable wolffian origin arising within the leaves of a broad ligament and, 5 years later, presenting with metastasis to the liver. The morphologic, immunohistochemical, ultrastructural, and DNA ploidy findings of the original and metastatic tumor, differential diagnoses, and the results of the English-language literature review are presented.     

Wolffian附件肿瘤3例并文献复习

目的探讨Wolffian附件肿瘤(Wolffian adnexal tumor,WAT)的临床病理学特征、鉴别诊断及其预后。方法对3例WAT进行临床病理学观察和免疫组化标记,并复习相关文献。结果 3例患者年龄分别为25、49及55岁,肿瘤分别位于后腹膜、左侧输卵管系膜及右侧卵巢。镜下肿瘤细胞呈弥漫实性或筛网状分布,弥漫实性区可见成片稍肥胖的梭形或卵圆形细胞及紧密排列的管状结构,管腔内衬立方或柱状上皮;瘤细胞无明显异型,核分裂罕见;管周有PAS阳性的基膜样物质。免疫表型:3例瘤细胞CKpan、vimentin、Calretinin均阳性,CD99阳性1例,α-inhibin阳性2例,PR、CK7、EMA均阴性。结论 WAT是一种罕见的具有低度恶性潜能的肿瘤,需进行长期密切随访。     

Endometrioid carcinoma with a low-grade spindle cell component: a tumor resembling an adnexal tumor of probable Wolffian origin

Endometrioid carcinoma is known to have many histopathologic variants, which may cause diagnostic difficulty. One rare variant resembles Wolffian adnexal tumor (female adnexal tumor of probable Wolffian origin). This pattern can produce a significant solid component within the tumor. Once the true endometrioid nature of the tumor is recognized, the tumor can appear deceptively high grade by International Federation of Gynecology and Obstetrics grading criteria, which take into account the percentage of the tumor showing solid growth. The English-language literature on this variant is scant, and its behavior is not well documented. We present a case of ovarian endometrioid carcinoma with a Wolffian adnexal tumor pattern that recurred 19 years after the original surgery; and the patient continues to remain well without evidence of disease 1 year following her second surgery, that is, 20 years of indolent behavior. This long clinical course shows evidence for low-grade behavior for this tumor.     

Endometrioid carcinoma of the fallopian tube resembling an adnexal tumor of probable wolffian origin: A case of report and review of the literature

Endometrioid carcinoma is a common tumor of the female genital tract, mainly affecting the uterus corpus and ovary. In the fallopian tube, endometrioid carcinoma is relatively uncommon. The female adnexal tumor of probable wolffian origin (FATWO), although occasionally seen elsewhere, is most typically encountered in the broad ligament. Endometrioid carcinoma of the fallopian tube resembling FATWO is a rare type of endometrioid carcinoma, and only 20 cases have been reported to date. Here, we report a case of endometrioid carcinoma of the fallopian tube resembling FATWO and review the literature.     

A case of endometrioid carcinoma of the fallopian tube mimicking an adnexal tumor of probable Wolffian origin.

We report a very uncommon case of endometrioid adenocarcinoma of the fallopian tube that mimicked, based on histology, a female adnexal tumor of probable Wolffian origin (FATPWO). We present our microscopic and immunohistochemical findings, and a review of the literature concerning these two entities. The differential diagnosis can be of great consequence, owing to the very different prognoses of the two tumors, and is based mainly on macroscopic appearance and immunohistochemical profile: epithelial membrane antigen (EMA) and CA125, generally lacking in FATPWO, are expressed in endometrioid adenocarcinoma, thus indicating the müllerian origin of this tumor.     

Endometrioid carcinoma of the fallopian tube resembling a female adnexal tumor of probable wolffian origin

Endometrioid carcinoma of the fallopian tube resembling a female adnexal tumor of probable wolffian origin (FATWO) is a rare, recently delineated, low-grade carcinoma that may be confused with both FATWO and high-grade carcinoma. Only about 20 cases have been reported so far, but it probably represents almost half of the endometrioid carcinomas of the fallopian tube. It has a better prognosis than conventional tubal endometrioid and serous carcinomas. Macroscopically, the tumors form a small, solid, polypoid mass that is usually confined within the tube. Microscopically, the tumors are characterized by proliferations of spindle cells in whorls and closely packed confluent elongated glands with focal tubular, sieve-like, and papillary structures. Unlike FATWO, this variant of endometrioid carcinoma exhibits focal squamous differentiation, intraluminal mucin, mild to moderate nuclear atypia, and occasional mitosis. Unlike FATWO, tumor cells are negative for inhibin-alpha and calretinin.     

Intranasal pericytic tumors (glomus tumor and sinonasal hemangiopericytoma-like tumor): report of two cases with review of the literature

An intranasal glomus tumor and a sinonasal hemangiopericytoma-like tumor are reported. Both patients were elderly women suffering from nasal bleeding, and presented with a polypoid mass arising in the nasal septum. Microscopically, the glomus tumor displayed a proliferation of uniform rounded or cuboidal epithelioid cells arranged in sheets and interrupted by a rich vasculature with a characteristic configuration mimicking the normal glomus bodies, while the sinonasal hemangiopericytoma-like tumor featured a perivascular proliferation of spindle- to oval-shaped cells that were arranged in short fascicles. Both tumors shared immunohistochemical features supporting their myoid differentiation by the expression of vimentin, alpha-smooth muscle actin and muscle-specific actin, albeit with no immunoreaction to desmin. Both the intranasal glomus tumor and sinonasal hemangiopericytoma-like tumor are characterized by a perivascular growth pattern and myoid differentiation, having a close relation to the 'perivascular myomas', which was recently designated.     

Tumor to tumor metastasis: report of two cases and review of the literature

Tumor-to-tumor metastases are uncommon. The most frequent donor tumor is lung cancer, while renal cell carcinoma (RCC) is by far the most common recipient. In this report, a carcinoma of the uterine cervix metastasizing to an RCC and a urothelial carcinoma of the urinary bladder metastasizing to a solitary fibrous tumor of the pleura are described. No similar cases have been found in the accessible literature. These cases are discussed and the findings are correlated with the data of the literature.     

Female adnexal tumours of probable Wolffian origin: an immunohistochemical study comparing tumours, mesonephric remnants and paramesonephric derivatives

AIMS: To establish an immunohistochemical profile of presumed female adnexal mesonephric tumours (FATWO) for diagnostic purposes and to compare the findings with those of mesonephric and paramesonephric derivatives in order to establish supportive evidence for a mesonephric origin. METHODS AND RESULTS: Standard immunohistochemistry was performed on formalin-fixed tissues. Tumours, mesonephric remnants and paramesonephric structures generally show positive staining for vimentin, CAM 5.2 and cytokeratins 7 and 19 but are negative for CK20 and 34 beta E12. EMA is positive in both mesonephric and paramesonephric derivatives but is negative in the tumours. Glutathione S-transferase mu (GST mu) is generally positive in both tumours and mesonephric derivatives but negative in paramesonephric structures. CONCLUSIONS: Immunohistochemistry plays little part in the diagnosis of FATWO. The tumours are generally cytokeratin and vimentin-positive and EMA-negative. GST mu, as a marker for the mesonephric duct, is a useful adjunct. Our findings of the study support but do not prove that FATWOs are of mesonephric origin.     

Primitive myxoid mesenchymal tumor of infancy: report of two cases and review of the literature

Primitive myxoid mesenchymal tumor of infancy is a recently recognized soft tissue tumor with only a few cases reported. Here, we reported another two cases of the lesion, a 5-month-old boy presenting with a soft tissue mass in the neck region that recurred 2 months later and a 3-day-old girl with a congenital superficial dorsal lumbar mass that extended to the spinal canal 1 month later. They shared similar histological patterns, such as unusual diffuse myxoid background, delicate vascular network, small cystic spaces, low to moderate cellularity, and primitive mesenchymal tumor cells. Immunohistochemically, the tumor cells showed positive for vimentin, CD99, CD117 and nestin, negative for myoid, lipoblastic, histiocytic, and neural markers. In conclusion, primitive myxoid mesenchymal tumor of infancy is a distinctive entity with its own clinical pathological features. Expression of CD99, CD117 and nestin may be consistent with the primitive nature of the tumor and may serve as ancillary markers for differential diagnosis from the other infantile tumors.     

软骨发育不全二例报告及文献复习

目的研究软骨发育不全的临床、影像学和病理学表现。方法复习1447例尸检中诊断为软骨发育不全的2例作回顾性研究。结果软骨发育不全患者身体长度基本正常而肢体较短,短肢以臂、大腿肢体近端为主。头颅增大,鼻梁塌陷,腰椎前凹,胸腔扁而小,肋骨异常的短。手指粗而短分开似＂三叉戟＂,肘生长受限。结论软骨发育不全是侏儒最常见的原因,在病理学上具有明显的特征性改变。     

Pigmented neurofibroma: report of two cases and literature review

Two cases of pigmented neurofibroma of the skin are reported. In case 1, the tumor was removed from the back of a 55-year-old man with no associated neurofibromatosis. In case 2, the tumor was removed from the abdominal wall of a 21-year-old woman with neurofibromatosis. Both tumors consisted of benign, short spindle cells and multiple foci of scattered melanin-laden cells. In case 1, the spindle cells were arranged in a storiform pattern, resembling features of dermatofibrosarcoma protuberans. Immunohistochemically, the spindle cells of both cases were demonstrated to be positive for S-100 protein and CD34. The melanin-laden cells stained positively for HMB-45. This report describes an additional two cases of pigmented neurofibroma that conform to the new diagnostic criteria for this disease.     

伸长细胞型室管膜瘤2例报道及文献复习

目的探讨伸长细胞型室管膜瘤的病理形态学特征。方法复习2例伸长细胞型室管膜瘤的临床病理资料,对手术切除的肿瘤组织,常规石蜡切片,HE染色,光镜观察,同时进行免疫组织化学标记GFAP、EMA、S-100蛋白、CKpan、Syn、CD34。结果例1位于腰2～3椎管内终丝,临床上主要表现出脊髓压迫症状,如运动及感觉障碍;例2发生于侧脑室,主要表现为头痛、呕吐、视物模糊。病理组织学上肿瘤富于细胞,排列成束状、交织状,细胞胞突细长双极,核异型性小,少数肿瘤细胞围绕血管周围形成室管膜瘤的无核区结构。免疫组化显示肿瘤细胞表达GFAP、EMA、CD99,而S-100蛋白阴性。结论伸长细胞型室管膜瘤是室管膜瘤的一种罕见亚型,病理诊断时尤其应注意与椎管内的神经鞘瘤和星形细胞瘤鉴别。