Biomedical subjects

Hector L Monforte

Publications and source records attributed to Hector L Monforte.

6 recordsLinked to original sources

PubMed2005

Surgical management of perineal masses in patients with anorectal malformations.

BACKGROUND: The aim of this study was to review the outcome of surgical management of various types of perineal masses encountered in patients with anorectal malformations (ARM). METHODS: Retrospective review from 2 large pediatric anorectal referral centers. RESULTS: Twenty-two patients with a perineal mass were identified in more than 2000 patients treated for an ARM over a 15-year period. The 22 patients (4 men) represented all levels of severity of ARMs. The lesions were of 3 types: lipomas (n = 10), vascular anomalies (n = 4), and hamartomas/choristomas (n = 8). The lipomas were carefully removed from between the muscle fibers during the posterior sagittal anorectoplasty. The vascular anomalies (3 of 4 were hemangiomas) underwent magnetic resonance imaging preoperatively, but none were found to invade deeply and all were excised at the time of the posterior sagittal anorectoplasty. The hamartomas/choristomas all occurred in women, and 50% arose as a pedunculated mass from the vulva. The lesions contained tissues such as glia, osteoid, nephrogenic rests, and endocervical-type mucosa. One was initially misinterpreted as a teratoma, prompting a wider excision. This and all subsequent patients have been correctly diagnosed pathologically as having either hamartomas or choristomas, which were not widely excised. Follow-up ranges from 5 months to 12 years. Six of the 10 lipoma patients are continent. One vascular anomaly was re-excised and there was minor wound separation in another. None of the hamartoma/choristoma lesions recurred. CONCLUSION: The presence of unusual perineal masses can add to the complexity of ARMs; however, most of these lesions can be carefully excised with preservation of the muscle complex and ultimate continence. Hamartomatous lesions can be mistaken for teratomas but do not require aggressive excision with clear margins.

Anal Canal↗

PubMed2004

Extralobar pulmonary sequestration presenting with torsion.

The current report describes the case of a 13-year-old girl with an extralobar pulmonary sequestration who presented with chest and back pains without evidence of infection. Her symptoms were discovered to be secondary to torsion and infarction of the pulmonary sequestration. Although pulmonary sequestration is not an uncommon differential diagnosis for chest masses, it is rare for it to present in this manner.

Adolescent↗

PubMed2004

Ewing sarcoma/peripheral primitive neuroectodermal tumor: adult abdominal tumors with an Ewing sarcoma gene rearrangement demonstrated by fluorescence in situ hybridization in paraffin sections.

The differential diagnosis of small round cell tumors is exhaustive and requires ancillary studies. Relatively recently, fluorescence in situ hybridization (FISH) using probes for specific gene rearrangements has gained wide acceptance. This technique is particularly useful in the differential diagnosis of Ewing sarcoma/primitive neuroectodermal tumor (ES/PNET) and desmoplastic small round-cell tumor (DSRCT). In ES/PNET, the EWS gene is juxtaposed to the FLI-1 gene in 85% of cases and to the ERG gene in another 7% of cases; the EWS gene is juxtaposed to the WTI gene in DSRCT. Documentation of the EWS gene rearrangements in EWS/PNET has previously been demonstrated in frozen tissue. We report 2 unusual cases of EWS/PNET diagnosed in abdominal tumors in adults. Although the immunohistochemical results supported a diagnosis of ES/PNET, 1 case morphologically resembled DSRCT. The diagnosis in these 2 cases was confirmed by the FISH demonstration of EWS/FLI-1 gene fusion in paraffin-embedded tissue. Thus, the usefulness of FISH demonstration of an EWS gene rearrangement with these specific probes in such unusual cases is supported and is demonstrated in paraffin-embedded tissue.

Abdominal Neoplasms↗

PubMed2003

The gastric cardia: to be or not to be?

The origin and biologic significance of cardiac gastric mucosa are controversial. Traditionally, it has been considered native mucosa and part of normal foregut development. It has been recently suggested that cardiac mucosa is present only as a metaplastic response to gastroesophageal reflux disease and therefore always abnormal. We evaluated the esophagogastric junction in 100 pediatric autopsy samples to determine the existence, characteristics, and length of pure cardiac mucosa at different ages. No patient had a history of gastroesophageal reflux disease. Cardiac mucosa immediately distal and contiguous to the esophageal squamous mucosa was identified in all 100 samples, varying in length from 0.1 to 3 mm; the mean length was 1 mm. There was an inverse correlation between patient age and length of cardiac mucosa; gender had no influence on measured length. Three patients had mild to moderate histologic esophagitis; two had gastritis. No metaplastic features or Helicobacter pylori were identified. These findings support the concept that there is a normal, variably narrow developmental zone at the esophagogastric junction covered by cardiac mucosa and is present at birth. When cardiac type mucosa is found in biopsy material, it does not necessarily represent evidence of a mucosal metaplastic response to gastroesophageal reflux disease.

Adolescent↗

PubMedDate not supplied

Maternal pulmonary adenocarcinoma metastatic to the fetus: first recorded case report and literature review.

We report the first maternal pulmonary adenocarcinoma metastatic to the fetus as well as an updated literature review. Review of the literature revealed that there have been only 67 cases of maternal malignancy metastatic to the products of conception. These were mostly malignant melanoma and hematopoietic tumors. A 46-year old multiparous woman with metastatic pulmonary adenocarcinoma, diagnosed at 23 weeks gestation, delivered a male infant who appeared normal at birth. The mother died 2 days after delivery. The child developed multiple scalp tumors a 2 weeks of age. The tumors recurred rapidly after initial resection. Wide local excision of the involved scalp and skin graft coverage was performed at 14 weeks of age. Histopathology of these tumors was identical to that of the maternal tumor. The maternal origin of these tumors was confirmed by fluorescence in situ hybridization (FISH). The child is now 5 years old and free of disease.

Adenocarcinoma↗

PubMedDate not supplied

Successful multimodality therapy of recurrent multifocal juvenile granulosa cell tumor of the ovary.

Juvenile granulosa cell tumor (JGCT) of the ovary, a rare pediatric cancer, carries a very poor prognosis in advanced and recurrent cases. A 10-year-old girl with stage IA JGCT, initially treated with resection only, presented with extensive unresectable multifocal pelvic recurrence. She underwent surgery, chemotherapy (cisplatin/paclitaxel alternating with cisplatin/cyclophosphamide/etoposide/bleomycin), myeloablative chemotherapy (carboplatin/etoposide/melphalan) with autologous bone marrow transplant, and pelvic radiation. She tolerated therapy well and is in complete remission 69 months after her recurrence.

Antineoplastic Combined Chemotherapy Protocols↗