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Klippel-Trenaunay syndrome (KTS) is an uncommon congenital angiodysplasia that manifests in infancy and is characterized by venous and lymphatic malformations of the skin, soft tissue, and bone causing limb hypertrophy. We report 2 patients with long-term KTS who developed lethal complications from uncommon and unusual manifestations. The 1st patient was a female with KTS who at 2 years of age underwent a below-the-knee amputation for a massively hypertrophied and malformed left foot. Two years later she required additional surgical removal of vascular malformations involving her left calf with extension to the groin, pubis, and ipsilateral abdomen. Fifteen years later she underwent splenectomy (400 g) revealing multifocal, cystically dilated vascular channels distorting the splenic architecture and died suddenly of massive intra-abdominal hemorrhage on the 2nd postoperative day. The 2nd patient was a 72-year-old male with long-standing KTS who presented with debilitating chronic penile and scrotal edema. Surgical excision of his lymphedematous scrotal and penile skin revealed a low-grade angiosarcoma arising in the setting of chronic lymphedema. The patient died shortly after surgery from massive hemorrhage due to traumatic rupture of malformed leg vessels. KTS may lead to significant morbidity and mortality, and pathologic consequences from long-term KTS have been rarely reported. These cases illustrate the risk of lethal hemorrhage, organomegaly from protracted vascular malformation, and development of vascular neoplasia associated with chronic lymphedema in KTS.

Objectives: Our study examined accumulated data on vaginal cytology follow-up results of women treated for endometrial carcinoma (EmCa) by hysterectomy in a large academic women’s hospital practice. Methods: This study analyzed cases of EmCa treated by hysterectomy from January 2006 to December 2008 with documented follow-up vaginal cytology and/or vaginal biopsy results available prior to September 2013. Results: Of 607 patients with EmCa who had documented hysterectomy findings, 420 had either follow-up vaginal biopsy or cytology results during a mean follow-up period of 42.5 months, and 414 (98.6%) had at least one vaginal cytology follow-up result. Of 414 patients followed with vaginal cytology results, 48 (11.6%) had biopsy results while 366 (88.4%) had only vaginal cytology results. Recurrent EmCa in the vagina was documented by biopsy in 11 patients, eight within 2 years of hysterectomy. Cytology findings of neoplastic glandular cells triggered diagnostic vaginal biopsies in two of 11 cases. Conclusions: Vaginal recurrences of EmCa were diagnosed in 11 (2.6%) of 420 patients with documented vaginal follow-up testing. Abnormal vaginal cytology findings directly led to vaginal biopsy diagnoses of EmCa in two of the 11 patients. Vaginal cytology is no longer a recommended routine surveillance option for detection of vaginal cuff recurrence of EmCa.

Cardiac calcified amorphous tumor is a rare pseudoneoplastic intracavitary mass composed of calcium deposits in a background of amorphous degenerating fibrin. We report occurrence of this lesion in the right atrium in two male patients. These were accompanied by calcific occlusion of the inferior vena cava and right pulmonary artery.

To examine whether women who have had a hysterectomy for cervical cancer may be at an increased risk of vaginal epithelial lesions.We studied 147 patients with invasive cervical carcinoma (76 squamous cell carcinomas [SCCs], 60 adenocarcinomas [ADCs], and 11 adenosquamous cell carcinomas) who were treated by hysterectomy and had vaginal pathologic follow-up for a mean period of 43.3 months.Of the patients, 15.0% (22/147) developed vaginal intraepithelial neoplasia (VAIN) or recurrence after hysterectomy, including two recurrent carcinomas and eight high-grade VAINs. More important, these high-grade VAINs or recurrent carcinomas were detected only in patients with cervical SCC within the first two years after hysterectomy but not in patients with cervical ADC. Eleven (23.4%) of 47 patients had at least one positive high-risk human papillomavirus (hrHPV) testing result during the follow-up period, and VAIN was detected in 54.5% (6/11) of patients with an hrHPV-positive result compared with 16.7% (6/36) with an hrHPV-negative result.Our results indicate that women with cervical cancer are at an increased risk of VAIN besides recurrence, and women with cervical SCC are more prone to high-grade VAIN/recurrence, especially within the first two years after hysterectomy. The significantly increased detection rate of VAINs/recurrence in the hrHPV-positive group suggests vaginal cytology and HPV cotesting might be the preferred method for surveillance in these women.

Systematic studies on Alzheimer's disease (AD)-related pathology that complement clinical and epidemiological data on dementia from low and middle income countries are rare. We report the first large study on AD-related pathology in autopsy service-derived brains from an urban center in India, a low/middle income country, and compare findings with a similar sample from New York. Amyloid-β plaques and neurofibrillary tangles were assessed in 91 brain specimens derived from hospital autopsy cases from Mumbai, India (age 60+ years; mean age 71.1 years, ± 8.3 SD; range 60-107 years) and compared with identically examined age-matched sample obtained in New York. These cases had no known clinical history of dementia. Our study showed that in comparison with the New York sample, the mean brain weight of the Mumbai sample was lower (p = 0.013) and mean diffuse plaque density was higher (p = 0.019), while differences in mean density and counts of neurofibrillary tangles and neuritic plaques were not statistically significant (p > 0.05). Our findings indicate that the burden of AD-related pathology was approximately equivalent in Mumbai and New York samples, which is at variance with expected lower AD-related lesion burden based on the clinical/epidemiological studies suggesting lower prevalence of AD in India.

Medullary carcinoma (MC) of the colorectum is a relatively new histological type of adenocarcinoma characterized by poor glandular differentiation and intraepithelial lymphocytic infiltrate. To date, there has been no epidemiological study of this rare tumor type, which has now been incorporated as a separate entity in the World Health Organization (WHO) classification of colorectal cancers. We used the population-based registries of the Surveillance, Epidemiology and End Results (SEER) database to identify all cases of colorectal MC between 1973 and 2006 and compared them to poorly and undifferentiated colonic adenocarcinomas (PDA and UDA, respectively). We observed that MCs were rare tumors, constituting approximately 5-8 cases for every 10,000 colon cancers diagnosed, with a mean annual incidence of 3.47 (+/-0.75) per 10 million population. Mean age at diagnosis was 69.3 (+/-12.5) years, with incidence increasing with age. MCs were twice as common in females, who presented at a later age, with a lower stage and a trend towards favorable prognosis. MCs were extremely rare among African-Americans. MCs were most common in the proximal colon (74%), where they present at a later age than the sigmoid colon. There were no cases reliably identified in the rectum or appendix. Serum carcinoembryonic antigen levels (CEA) were elevated prior to first course of treatment in 40% of the patients. MCs were more commonly poorly differentiated (72%), with 22% being undifferentiated. MCs commonly presented with Stage II disease, with 10% presenting with metastases. Only one patient presented with N2b disease (>7 positive nodes). Early outcome analyses showed that MCs have 1- and 2-year relative survival rates of 92.7 and 73.8% respectively. Although MCs showed a trend towards better early overall survival, undifferentiated MCs present more commonly with Stage III, with comparatively worse early outcomes.