Acute disseminated encephalomyelitis in China, Singapore and Japan: a comparison with the USA.

BACKGROUND AND PURPOSE: Ethnicity-related differences in the incidence of acute disseminated encephalomyelitis (ADEM) and other demyelinating diseases including multiple sclerosis and neuromyelitis optica spectrum disorders have been reported. Little is reported on the influence of ethnicity and geographical location in ADEM. METHODS: Medical records of patients who presented with ADEM (ICD-9 323.61 and 323.81) at large referral hospitals in China, Singapore and Japan (years 1992-2015) were retrospectively reviewed and data were collected in a centralized database. Presenting features and outcomes of ADEM were compared between this multi-country Asian cohort and a uniformly collected US cohort using risk differences and risk ratios. Both cohorts were standardized to a 35% pediatric population to facilitate the comparison. RESULTS: There were 83 Asian patients (48 male, 16 pediatric) followed for a median of 2 (25th-75th percentile 1-10) months. Asian patients exhibited a 26% higher prevalence of spinal cord involvement on magnetic resonance imaging [95% confidence interval (CI) 0-52%; P = 0.05; 63% vs. 37%], a 39% lower prevalence of preceding events (95% CI 12-65%; P < 0.01; 33% vs. 72%) and a 23% lower prevalence of corpus callosum involvement (95% CI 7-39%; P < 0.01; 8% vs. 31%). No difference was observed between the two cohorts in the probability of relapse over the first year after disease onset. CONCLUSIONS: It is hypothesized that the high proportion of Asian patients with spinal cord lesions relates to genetic vulnerability or the higher incidence of neuromyelitis optica spectrum disorders in Asia or could be a spurious association. ADEM presentations most probably vary across geographical settings or ethnicities.

Cervical schistosomiasis, human papilloma virus (HPV), and human immunodeficiency virus (HIV): a dangerous coexistence or coincidence?

BACKGROUND: Female genitourinary schistosomiasis (FGS) is widespread in endemic areas causing significant morbidity and mortality. Recent data suggest that FGS of the cervix not only is considered a risk factor for contracting different sexually transmitted diseases (STD), but also plays a significant role in modifying the natural history and immunological response to those infections, in particular HIV and HPV. CASE REPORT: A 32-year-old female from Zambia, who was recently diagnosed with HIV and high-grade dysplasia with koilocytosis on cervical Pap smear, underwent cervical conization which confirmed moderate cervical dysplasia and also revealed the presence of viable and nonviable schistosoma eggs in cervical stroma. Four different HPV types were isolated by PCR, including one "low-risk" (type 6) and three "high-risk" types (types 45,56, and 58). CONCLUSION: The presence of HPV, HIV infection, and cervical schistosomiasis in our patient is likely more than coexistence of multiple agents in the same milieu as cervical schistosomiasis increase susceptibility for other STDs including HIV and HPV. Therefore, in patients with schistosomiasis, immediate treatment for schistosomiasis and additional testing for HIV and HPV is warranted.

Serous tumor of low malignant potential of the fallopian tube.

Although serous tumors of low malignant potential (STLMP) of the ovary are relatively common, similar tumors of the fallopian tube are exceedingly rare. We report the case of an STLMP of the fallopian tube in a 31-year-old woman and review the literature.

Atypical presentation of microscopically advanced ovarian carcinoma.

Epithelial ovarian cancer (EOC) continues to be an academically challenging and clinically problematic disease. Even with recent advances, the overall 5-year survival is still 31% to 42% in various studies. Deaths from EOC outnumber those due to cervical, vulvar, and endometrial carcinomas combined. Screening for EOC has shown limited success in early detection. The Pap smear is not a dependable tool in EOC screening, though at times it can be the first evidence of ovarian disease. We report a case of EOC that was diagnosed during evaluation of an abnormal Pap smear. On completion of evaluation, stage IIIA endometrioid-type adenocarcinoma of the ovary was diagnosed. Occult EOC should be considered in patients with abnormal findings on cervical cytology after cervical and uterine carcinomas are ruled out.

Lymphoepithelioma-like carcinoma of the uterine cervix.

We describe two rare cervical tumors having morphologic features closely resembling those of the nasopharyngeal lymphoepithelioma. This entity has historically been classified as a subtype of squamous cell carcinoma, but after reviewing the literature and the two cases presented here, we propose that this tumor is a distinct carcinoma of the cervix that differs from squamous cell carcinoma in that it carries a more favorable prognosis, typically affects a younger population of women, is more prevalent in noncaucasian populations (especially those of Asian descent), and lacks a clearly defined association with infection due to human papilloma virus (HPV).

Uterine arteriovenous malformation necessitating hysterectomy with bilateral salpingo-oophorectomy in a young pregnant patient.

This case report describes a 31-year-old woman at 8 weeks' gestation with large arteriovenous malformation of the uterus involving bilateral uterine and ovarian arteries. She had a history of multiple pregnancy losses, as well as spontaneous copious vaginal hemorrhage. The patient underwent an embolization procedure followed by total abdominal hysterectomy and bilateral salpingo-oophorectomy. The uterus was very small (30 g) despite its gravid status, and the overall microscopic findings indicated Müllerian system hypoplasia in addition to vascular malformation.

Basal cell carcinoma of the vulva. A report of four cases.

BACKGROUND: Basal cell carcinoma is the most common malignant tumor of the skin, with approximately 400,000 new cases yearly in the United States. Basal cell carcinomas account for 2-3% of all vulvar malignancies. CASES: Four patients presented in the eighth and ninth decades of life (70, 78, 87 and 89 years). Seventy-five percent of patients had a unifocal lesion. Cases 3 or 4 presented with recurrent lesions at 5 and 10 years, respectively. All patients were treated with wide local excision. Surgical margins were free of disease. None of the patients had lymph nodes suspicious for malignancy. CONCLUSION: Basal cell carcinoma can present as a unifocal or multifocal lesion. The lesions are usually located on the labia majora. Patients are frequently diagnosed with basal cell carcinoma of the vulva in the eighth and ninth decades of life. Treatment consists of wide local excision. Although 50% of these cases recurred, the lesions were reexcised, with wide local resection. No metastatic lesions were identified in any of the patients.

Leiomyomatosis peritonealis disseminata: clinicopathologic analysis of five cases.

Leiomyomatosis peritonealis disseminata (LPD) is an exceedingly rare, usually benign condition that clinically simulates a disseminated malignancy. It occurs predominantly in women of childbearing age. Approximately 50 cases of LPD have been described in the literature with 2 cases reported to have progressed into aggressive leiomyosarcomas. Excessive hormonal stimulation appears to correlate with LPD development since it is commonly associated with pregnancy. Conservative treatment with long-term follow-up is recommended because of LPD's generally indolent clinical course. We report 5 cases of LPD with a review of the literature.

Basal cell carcinoma due to radiotherapy for carcinoma of the cervix.

We report a case of basal cell carcinoma arising in a previously radiated field after treatment of cervical cancer. Our search of the literature yielded no other case of basal cell carcinoma due to irradiation for cervical carcinoma.

Dracunculosis of the broad ligament. A case of a "parasitic leiomyoma".

This report describes the case of a 29-year-old Nigerian woman with dracunculosis presenting as an adnexal mass. The patient had a history of two second-trimester spontaneous abortions for which she was being evaluated. During the evaluation, she was found to have multiple uterine leiomyomas and a calcified left adnexal mass by pelvic ultrasound and radiography. At laparotomy, the calcified mass was excised from the left broad ligament, and a myomectomy was performed. Histologic examination revealed a calcified, coiled guinea worm embedded in the fibrous tissue of the broad ligament. Only one other case of calcified guinea worm in the broad ligament has been reported in the literature.

Primary adenocarcinoma of the vulva, possibly cloacogenic. A report of two cases.

Primary adenocarcinoma of the vulva, arising in continuity with the epidermis and unrelated to the underlying mucus glands, is a distinctly uncommon neoplasm. The origin of such an unusual tumor, although attributed to cloacal remnants, remains speculative. We describe two patients with this rare lesion. No other primary tumor was found in either of the two after a thorough investigation. One patient was treated with radical vulvectomy and was free of disease for 10 years, until her death from unrelated causes. The second patient was treated with wide local excision and was free of disease four years postoperatively. Our experience indicates the need to recognize this tumor as a primary and not metastatic neoplasm of the vulva. We also noticed that in both cases, despite its histologic malignancy and ulceration, the tumor was superficial in its extent and without any tendency for deeper invasion. It can therefore be treated effectively with wide local excision, thus eliminating the need for radical vulvectomy.

Ovarian myxoma: ultrastructural and immunohistochemical findings.

The ultrastructural and immunohistochemical findings are reported in two ovarian myxomas, one of which was also associated with a sclerosing stromal tumor of the same ovary. Both neoplasms showed a myxoid, moderately cellular proliferation of spindle and stellate cells interspersed with areas of fibrosis and hemorrhage as well as delicate vascular spaces. Ultrastructurally, stellate neoplastic cells with irregular nuclei and occasional nucleoli were embedded in a mucinous and loose collagen matrix. Their cytoplasm showed abundant intracytoplasmic thin filaments that rarely condensed into poorly formed dense bodies. These thin filaments correlated with immunoreactivity for muscle-specific actin and vimentin. The neoplastic cells showed no immunoreactivity with antibodies to desmin, S-100 protein, cytokeratin AE1:AE3, factor VIII-related protein, or placental alkaline phosphatase. These ultrastructural and immunohistochemical findings are consistent with myofibroblastic differentiation. These ancillary studies exclude important, clinically more aggressive differential considerations such as myxoid rhabdomyosarcoma, myxoid liposarcoma, myxoid neural tumors, mucinous adenocarcinoma showing pseudomyxomatous change, and myxoid-appearing endodermal sinus (yolk sac) tumor.

Primary fallopian tube carcinoma with coexistent tuberculous salpingitis: a case report.

A case of primary carcinoma of the fallopian tube with coexistent tuberculosis has been presented, and previously reported cases in the literature reviewed. These cases represent a rare occurrence where the diagnosis might be missed without a thorough pathologic evaluation. Clinicians should maintain an index of suspicion for fallopian tube carcinoma in perimenopausal or postmenopausal women with menometrorrhagia who fail to respond to hormonal therapy and a D and C. The finding of a tuboovarian abscess in a postmenopausal woman should also cause concern for tubal carcinoma, as it is usually a disease of menarcheal women. Similarly, a possibility of granulomatous salpingitis should always be borne in mind in the differential diagnosis of inflammatory diseases of the fallopian tube.

Paratesticular myxoma: an unusual benign intrascrotal neoplasm.

We have presented a case of paratesticular myxoma and have described the clinical history, findings on light microscopy and immunohistochemistry, and possible pathogenesis. Although primary paratesticular myxoma is a rare lesion, it should be considered in the differential diagnosis of intrascrotal mesenchymal tumors.

The Sister (Mary) Joseph nodule: its significance in gynecology.

Twenty-five cases of metastatic carcinoma of the umbilicus in women diagnosed and treated at Emory University Hospitals from 1973 to 1989 were reviewed, as were all previously reported cases in the English language literature. In our series, overall survival was greatest in patients treated with aggressive surgery and chemotherapy, compared with patients receiving no therapy or those treated with either surgery or adjunctive therapy alone. Individual survival was enhanced when the primary tumor site was of ovarian origin.

Early diagnosis of vulvar neoplasia as a result of vulvar self-examination.

Vulvar self-examination was used to facilitate early diagnosis and treatment of vulvar neoplasia in eight patients. Five of them were found to have invasive squamous cell carcinoma of the vulva, two had carcinoma in situ, and one had a vulva melanoma. All eight patients benefited from early diagnosis, which allowed definitive treatment and vulvar conservation.

Granular cell tumors of the vulva.

Granular cell tumor (GCT), although nearly ubiquitous, is seen infrequently in the vulva. A review of the surgical pathology files from Grady Memorial Hospital, Atlanta, Georgia, from 1983 through 1987 identified eight cases of vulvar GCT. Five of the eight patients had more than one skin and soft tissue lesion. Two of the five had biopsy-proven multicentric GCT with a unique clinical course. One of the patients was a 32-year-old woman with multiple vulvar, lingual, laryngeal, bronchial and pulmonary GCT, necessitating multiple excisions and ultimately pneumonectomy. The second patient had multiple GCTs in the vulva and inguinal area and finally in both lungs, resulting in her death at age 39. No dependable microscopic features could be identified to distinguish benign GCT from its more aggressive variant. However, Feulgen DNA histomorphometry demonstrated aneuploidy in the patient with apparent lung metastases, whereas the tumors from patients with a benign course as well as from the patient with multiorgan involvement were diploid. In three of five patients who could be interviewed there was a history of soft tissue tumors in members of the family. The multifocal nature and possible familial component of GCT need to be explored further.

Immature teratoma of the fallopian tube. A case report.

An immature cystic teratoma of the left fallopian tube was found incidentally in a 30-year-old, multiparous woman requesting tubal ligation. Only one case of immature tubal teratoma was reported on previously.