A Rare Instance of Chronic Masson's Tumor Presenting in the External Auditory Meatus and Surgical Plan: A Case Report.

Intravascular papillary endothelial hyperplasia (IPEH), or Masson's tumor, is a rare and benign proliferation of endothelial cells typically of vascular origin. Common locations of Masson's tumor include the head, neck, orbit, lip, pharynx, and mandible. It is typically seen in middle-aged adult life and females. Possible differential diagnoses include hemangioma, benign vascular formation, angiosarcoma, and neurofibromatosis. The exact pathophysiology of Masson's tumor is currently unknown. We present the case of a middle-aged 47-year-old male with a pure type of Masson's tumor presenting with pedunculated, malleable lesions across the posterior scalp and circumferential neck, on the pinna of the right ear, and within the right external auditory meatus. The lesions within the right external auditory meatus caused conductive hearing loss. The plan is a complete surgical excision without wide margins. The patient was referred to an ear, nose, and throat (ENT) surgeon due to the complicated location of the lesion within the external auditory meatus. This case serves as a differential diagnosis of conductive hearing loss complicated by Masson's tumor.

Multiple low-grade sarcomas of fibroblastic type in the setting of HIV and acquired epidermodysplasia verruciformis.

A 46-year-old white male with a history of HIV (CD4 245), acquired epidermodysplasia verruciformis, anal carcinoma in situ, hepatitis B and C presented with 3 asymptomatic, nontender, firm pink/skin-colored nodules involving the arm, left lateral leg, and right third finger. One year later, he developed a similar lesion on his right medial lower leg. Excisional biopsy of one of the lesions showed an atypical spindle cell neoplasm of the dermis compatible with a low-grade sarcoma of fibroblastic origin. Testing for human herpes virus-8, 23 human papillomavirus types, Epstein-Barr virus, and FUS fusion protein were negative. The patient underwent diagnostic imaging with computed tomography scans of the chest, abdomen, and pelvis along with positron emission tomography scan to ensure that there was no other occult primary tumor, all of which were negative. The lesions were excised and have not recurred with 3 years of follow-up. The best histopathologic term for these lesions is multiple low-grade sarcomas of fibroblastic phenotype. They have been proven to be nonaggressive, with little or no metastatic potential. This is a neoplastic process that has not been well defined in the literature. To our knowledge, there are no previous reports of these lesions occurring in multiple sites or in an HIV-positive patient.

Caudal dysplasia syndrome and sirenomelia: are they part of a spectrum?

Caudal dysplasia syndrome (CDS) is associated with hypoplastic lower extremities, caudal vertebrae, sacrum, neural tube, and urogenital organs. Sirenomelia is characterized by a single lower extremity, absent sacrum, urogenital anomalies, and imperforate anus. There is controversy in the medical literature about whether sirenomelia and CDS are part of the spectrum of the same malformation. Patients with CDS and sirenomelia were identified from our pathology files from 1991 to 2006. Maternal history, pathologic examination, and radiographs were collected and tabulated. We found 9 cases with CDS and 6 with sirenomelia. Fully 7 of 9 patients with CDS (77.7%) versus none of sirenomelic babies were infants of diabetic mothers. Congenital heart disease was present in 5 patients with CDS (55.5%) and none of the infants with sirenomelia. Of 9 children with CDS 2 (22.2%) had bilateral renal agenesis versus 66% of sirenomelics. Single umbilical artery was found in 33% of cases with CDS and 100% of children with sirenomelia. External genitalia were ambiguous in 2 of 9 patients (22.2%) with CDS and in all patients with sirenomelia. Imperforate anus was found in 10 cases (66.6%) divided as 4 of 9 babies with CDS (44.4%) and all patients with sirenomelia. Three patients with CDS had concomitant maternal diabetes mellitus and chronic hypertension. These babies also had cleft lip and palate. Congenital heart disease was found in 55.5% of cases with CDS and none of the children with sirenomelia. We conclude that although CDS and sirenomelia share many similar features, they are two different entities.

Angiosarcoma of the bladder: case report and review of the literature.

Our objective was to present a new case of angiosarcoma of the bladder after therapeutic radiation of the prostate, and discuss the treatment and clinical course of this rare tumor; the role of multimodality treatment is also discussed. We report a case of angiosarcoma of the bladder. Presentation, clinical course, and treatment were outlined and discussed. A MEDLINE search of all reported cases of angiosarcoma in the English language literature was performed. Thirteen previous cases of bladder angiosarcoma have been reported and three previous cases have been reported after therapeutic radiation. Hematuria was the most common presentation. Overall survival is poor, with 5-year survival rates at 35%. Longer-term survival has been demonstrated in patients who have had a multimodal approach to treatment, which combines radical surgery with chemotherapy and radiotherapy. Angiosarcoma of the bladder is a rare disease with overall poor prognosis. Optimal treatment has not been defined, but multimodality approaches appear to have a survival benefit.

Does histopathology predict parathyroid hypersecretion and influence correctly the extent of parathyroidectomy in patients with sporadic primary hyperparathyroidism?

BACKGROUND: Parathyroid histopathology has been used to predict single or multiglandular disease (MGD). "Hyperplasia" implies MGD, whereas "adenoma" suggests single gland involvement. Intraoperative parathyroid hormone (PTH) monitoring (IPM) guides parathyroidectomy based on function. We sought to evaluate the accuracy of histopathology in the diagnosis of single or MGD and in predicting operative success. METHODS: We reexamined the parathyroid glands from 402 patients with sporadic primary hyperparathyroidism (SPHPT) who underwent initial IPM-guided parathyroidectomies. Operative findings and outcome were correlated with histopathology of excised glands. Operative success was eucalcemia for >or=6 months and recurrence of hypercalcemia/high PTH after successful parathyroidectomy. RESULTS: Of 402 patients, 384 had 1 gland excised resulting in operative success; hyperplasia was diagnosed in 244 of the 384 (64%), with only 2 developing recurrence. Of the 384 patients, 140 (37%) had adenomas with 1 late recurrence. There were 18 patients with MGD (14 hyperplasias, 4 adenomas). There were 5 failures with hyperplasia predicting MGD. Histopathology was incorrect in predicting the number of glands involved in 249 of 402 (62%) patients, and IPM was incorrect in only 13 (3%). CONCLUSION: Histopathology of excised abnormal parathyroid glands does not predict the secretory function of the remaining parathyroid glands left in situ. IPM guided parathyroidectomy accurately based on function alone; however, histopathology was inaccurate in predicting MGD and should not be used to guide parathyroidectomy in patients with SPHPT.

Intestinal and multivisceral transplantation in children with severe gastrointestinal dysmotility.

BACKGROUND/PURPOSE: Severe gastrointestinal dysmotility (GID) impairs patients' quality of life and is almost uniformly fatal after complications of parenteral nutrition. Intestinal and multivisceral transplants have been used as alternative treatment of these disorders. We studied patients with GID treated with transplantation in our center, and reviewed their outcome to determine the therapeutic efficacy of multivisceral transplants. METHODS: The transplant database was searched for patients with GID from 1994 to 2001. We excluded patients with Hirschsprung disease, scleroderma, and diabetic enteropathy. We reviewed explanted organs, histochemistry, and immunohistochemistry and classified cases by etiology. RESULTS: We selected 12 children with GID from 124 patients transplanted. Nine presented before 1 year and 3 started with symptoms between 2 and 8 years. By combined clinical and histopathological features, 6 were classified as megacystis microcolon intestinal hypoperistalsis syndrome, 4 as chronic idiopathic intestinal pseudoobstruction, and 2 as intestinal neuronal dysplasias. Six patients died during the follow-up from 21 to 546 days after transplant. The Kaplan-Meier actuarial survival rates were 66.7% at 1 year and 50% at 3 years. CONCLUSIONS: Multivisceral transplantation is a valuable therapeutic alternative for children with severe GID who cannot be adequately managed with parenteral nutrition.

Giant aneurysm of the atrial septum associated with premature closure of foramen ovale.

Premature closure or restriction of foramen ovale (PCFO) is a rare congenital anomaly that can lead to a wide spectrum of cardiac malformations. This spectrum of secondary malformations appears to depend on the gestational timing of closure of the foramen ovale and to the degree of restriction. Earlier in the gestation, closure of the foramen has been associated with severe hypoplasia of the left ventricle whereas later closure has been associated with right heart failure and rarely with the formation of an aneurysm of the atrial septum. We describe the case of a 1 day old infant in whom PCFO resulted in severe right heart failure in addition to the formation of a giant atrial septal aneurysm.

Nonimmune hydrops fetalis in the liveborn: series of 32 autopsies.

Nonimmune hydrops fetalis (NIHF) or generalized soft tissue edema and cavity effusions may be due to cardiovascular diseases, congenital infections, genitourinary malformations, thoracic masses, placental conditions, chromosomal abnormalities, and idiopathic. We report 32 cases of NIHF from among 429 neonates who underwent autopsies (incidence 7.45%). Sixteen cases (50%) had cardiovascular disease; all were due to low output cardiac failure; 7 had structural congenital heart disease. Three of the children with congenital heart disease also had chromosomal abnormalities: 2 had trisomy 18 and 1 had Noonan syndrome. Among myocardial conditions were five subjects with cardiomyopathies (1 of each of the following types): oncocytic, dilated, endocardial fibroelastosis, cardiac glycogenosis, and carnitine deficiency; 3 had myocarditis, and 1 had cardiac rhabdomyomas. Congenital infections were due to cytomegalovirus in 3 cases, bacteria in 2, and parvovirus in 1. The mechanism of NIHF in these cases might be a combination of decreased myocardial contractility due to myocarditis and fetal anemia. Genitourinary diseases were present in 5 newborns: Two had congenital nephrotic syndrome, 1 had VACTER association, 1 had prune-belly syndrome, and 1 had urogenital sinus malformation. Intrathoracic lesions were found in 2 babies (pulmonary sequestration and diaphragmatic hernia). One twin died of volume overload due to twin transfusion syndrome. Only 2 newborns were classified as idiopathic. Our study shows that cardiovascular diseases that lead to heart failure or impaired venous return are more common in the liveborn (50%), whereas congenital infections are more common in the stillborn with NIHF.

Open lung biopsy in pediatric patients with respiratory failure after abdominal transplantation.

To understand the utility of open lung biopsy (OLB) in the evaluation of respiratory failure in pediatric abdominal organ transplant we reviewed the records of nine children in this patient population who underwent an OLB. Eight of nine patients had undergone a previous non-diagnostic bronchoalveolar lavage. Biopsies were performed at the bedside in the pediatric intensive care unit and tissue was processed by the Department of Pathology with special stains for infectious agents. There were no significant complications of OLB. A specific treatable etiology was identified in four patients (respiratory syncytial virus, adenovirus, graft-vs.-host disease and post-transplant lymphoproliferative disease), leading to a change in therapy and survival in two. Overall survival was 44%. Given the low morbidity, OLB as performed in this study appears appropriate in this patient population.

Neointimal hyperplasia on a cell-seeded polytetrafluoroethylene graft is promoted by transfer of tissue plasminogen activator gene and inhibited by transfer of nitric oxide synthase gene.

OBJECTIVE: The objective of this study was to examine the effect of tissue plasminogen activator (tPA) and endothelial nitric oxide synthase (eNOS) on thrombosis and neointimal hyperplasia on a polytetrafluoroethylene (PTFE) graft seeded with smooth muscle cells (SMCs). METHODS: SMCs retrovirally transduced with tPA and eNOS genes were seeded on PTFE grafts and then implanted into the infrarenal rabbit aorta. Thrombosis and neointimal hyperplasia on the grafts were examined after 30 and 100 days of implantation. RESULTS: At 30 days of implantation, thrombus was observed on the luminal surface of both unseeded and SMC seeded control grafts, whereas grafts seeded with SMCs secreting tPA were nearly free of thrombus. At 100 days, the neointima on grafts seeded with tPA transduced SMCs was significantly thicker (925 +/- 150 microm, n = 5) than neointima on the other grafts (range, 132 to 374 microm; P < .001). Neointima thickness on grafts seeded with eNOS transduced SMCs (154 +/- 27 microm) was similar to that of unseeded grafts (132 +/- 16 microm, P > .05); both were thinner than those on grafts seeded with SMCs transduced with only lacZ gene (287 +/- 35 microm). The ratio of seeded cells in the neointima was significantly higher on SMC/tPA grafts (46% +/- 8%) than SMC/NOS grafts (21% +/- 6%, P < .05), indicating tPA transduced cells proliferated more than eNOS transduced cells. CONCLUSIONS: Engineered tPA expression in seeded SMCs causes significantly more neointimal hyperplasia, despite the favorable inhibition of luminal thrombus. eNOS expression in the seeded cells inhibits neointimal hyperplasia.

A hydatidiform mole in a postmenopausal woman. A case report and review of the literature.

Gestational trophoblastic disease occurs in less than 1 per 1200 pregnancies in the United States. The spectrum of this disease ranges from benign hydatidiform mole to trophoblastic malignancy (placental-site trophoblastic tumor and choriocarcinoma). Benign gestational trophoblastic disease generally occurs in women of reproductive age and is extremely rare in postmenopausal women. To our knowledge, our case represents only the third description in the world literature of a benign complete hydatidiform mole in a woman with a history of amenorrhea greater than 1 year. We describe the case of a 61-year-old postmenopausal woman who underwent an emergent total abdominal hysterectomy due to uncontrollable vaginal bleeding associated with an increased serum beta-human chorionic gonadotropin level. The resected uterus contained an endometrial, cystic, grapelike tumor. Microscopic examination demonstrated hydropic degenerated villi with a circumferential trophoblastic cell proliferation and moderate atypia, consistent with a complete hydatidiform mole. The morphologic and immunophenotypic characteristics are presented, as well as the results of a literature review.

Primary malignant melanoma of the right colon.

The small and large intestines are the most common sites for metastases from cutaneous malignant melanoma. However, primary melanomas in these sites are exceedingly rare. There are several case reports of patients with primary melanoma of the small bowel, but finding of a solitary primary melanoma in the colon is exceedingly rare. We describe a patient that was operated on for bowel obstruction due to colonic intussusception resulting from a right colonic tumor. Histopathological examination confirmed a diagnosis of malignant melanoma. A thorough postoperative investigation did not reveal a primary lesion in any other site. Two years after surgery, there was no evidence for recurrent disease. The treatment and prognosis of metastatic and primary melanoma of the gastrointestinal tract is discussed as well as the embryonic base for development of primary malignant melanoma of the intestine. Primary malignant melanoma of the intestine is an extremely rare lesion that may arise in the large bowel as well. It must be differentiated from other intestinal tumors and mandates a thorough investigation to rule out the possibility of being a metastasis from another more common primary site.

Molar gestations and hydropic abortions differentiated by p57 immunostaining.

Classification of molar gestations into complete and partial and their differentiation from hydropic abortions traditionally are accomplished by morphology alone. The process sometimes may be inaccurate or inconclusive. With the availability of p57 immunostaining it may be possible to objectively classify these lesions. We used p57 for the differential diagnosis of hydropic abortions and molar gestations and correlated the findings with the clinical outcome of patients in each category. First, 86 cases were originally classified by histomorphology into hydropic abortion (42) and molar gestations (23 complete and 21partial). Based on the pattern of p57 staining the cases were reclassified into 45 hydropic abortions, 15 partial moles and 26 complete moles (3 cases with previous diagnosis of complete mole based on morphology were reclassified as hydropic abortion). Clinical follow-ups ranged from 6-24 months and showed persistent trophoblastic disease in 8 cases (31%) of complete moles and 3 cases (20%) of partial moles (p = 0.47). No hydropic abortion cases demonstrated persistent trophoblastic disease. One patient with partial mole developed choriocarcinoma. This study confirms that p57 objectively distinguishes hydropic abortions from molar gestations (partial and complete moles). This differentiation is clinically relevant since patients with hydropic abortions do not need to be followed while patients with molar gestations do.

Value of autopsy in nonimmune hydrops fetalis: series of 51 stillborn fetuses.

Nonimmune hydrops fetalis (NIHF) is used to describe fetuses and newborns with generalized edema and cavity effusions. It is helpful to alert physicians about the presence of anemia, heart failure, and/or hypoproteinemia, but this diagnosis is frequently overlooked. We reviewed the autopsy files from 1990 to 2000, selected all cases with NIHF including clinical information (with maternal laboratory tests and ultrasound), and classified patients by etiology. Among 840 stillborn autopsies during the 11-year period, we found 51 with NIHF (6.07%). The clinical summary had mentioned hydrops in 14 patients and the etiology in another 7 by fetal ultrasonography, but without addressing the possibility of hydrops. In the remaining 30 cases neither hydrops nor an etiology was mentioned. Other pertinent diagnoses were maternal diabetes mellitus (4), congenital heart disease (3), and cystic hygroma (2). The following diagnoses were made in one instance each: cardiac tumor, twin transfusion syndrome, congenital adenomatoid malformation, syphilis, Turner syndrome, and cerebral arteriovenous malformation. Postmortem and placental examination confirmed the following etiologies: congenital infections (17); placental pathology significant enough to explain NIHF (10); cardiovascular diseases (8) (further classified as congenital heart disease [3], rhabdomyoma [1], and vascular malformations [4]); chromosomal abnormalities (6); uncontrolled maternal diabetes (4); intrathoracic lesions (2); prune-belly syndrome (2); and idiopathic NIHF (2). Only 3.9% of the cases studied had no identifiable etiology. The cause of hydrops was confirmed by autopsy in 47 fetuses (92%), which further supports the importance of performing an autopsy. Thirty-two cases (62.74%) had placental abnormalities helpful to the etiology (parvovirus, syphilis, Turner's syndrome, etc.). In 20 instances, the clinical summary had no mention of either hydrops or any of the diseases leading to it. The autopsy in conjunction with placental examination and fetal ultrasound represent the best combination to determine the etiology of NIHF among stillborn fetuses.