Malignant thymoma in children: a 20-year review

Malignant thymomas are extremely rare in children, with only 27 cases reported thus far in the pediatric surgical literature. We report four additional cases diagnosed at this institution over the past 20 years (ages 3 to 14 years). Clinical presentations included superior vena cava syndrome, cough, dyspnea, cyanosis, enlarging mediastinal mass, spontaneous pneumothorax, and pleuropericardial effusion. Three patients underwent incomplete resection of the mass or biopsy because of "unresectability" and were treated with radiotherapy and adjunctive chemotherapy. One patient underwent near complete macroscopic resection as well as radiotherapy and chemotherapy. All patients died at intervals ranging from 6 months to 2 1/2 years after diagnosis. Three patients were found to have metastatic disease prior to death or at autopsy. In one case, the initial pathological diagnosis was lymphocytic thymoma. After ultrastructural studies were performed, the diagnosis was changed to thymic T-cell lymphoma. This patient subsequently developed acute lymphoblastic leukemia 3 months after surgical resection followed by radiotherapy. Malignant thymomas are highly aggressive tumors in children. A radical surgical approach with complete excision of the tumor and contiguous structures in continuity, with adjunctive radiotherapy and chemotherapy remains the only hope for survival in children with these rare lesions.     

Rectal duplications in children: two case reports and review of the literature

Duplications of the rectum are extremely rare, with only 60 cases reported in the world literature. We report two patients with cystic duplications of the rectum who underwent surgical intervention in our institution during the past 10 years. A 5-year-old white male presented with constipation and tenesmus as well as a 4-cm cystic mass that protruded from the anus when he strained to pass stool. Rectal examination and ultrasound suggested the clinical diagnosis of rectal duplication. Transanal submucosal resection of a 3-cm cystic mass adherent to the posterior rectal wall was carried out. Histologic examination revealed a rectal duplication containing zones of ciliated respiratory epithelium. The second patient, a 6-year-old white female, presented with a history of chronic constipation. A large, cystic presacral mass was detected by rectal examination and confirmed by CT scan. Complete submucosal resection of the cystic duplication was performed through a posterior trans-sacral approach. Histology revealed a rectal duplication lined by respiratory epithelium. Although duplications are generally lined by mucosa of the adjacent bowel, heterotopic mucosa, most commonly gastric or pancreatic, may be present. Rectal duplications with ectopic gastric mucosa have been previously described, but to our knowledge the presence of respiratory epithelium in a rectal duplication has never been reported. Complete resection of these lesions results in cure.Presented at the 21st Annual Meeting Canadian Association of Paediatric Surgeons Edmonton, Alberta, Canada September 21–23, 1989 Offprint requests to: A. L. Bensousson     

Desmoplastic small round cell tumor with primary ovarian involvement: case report and review

BACKGROUND: Desmoplastic small round cell tumor (DSRCT) is a rare, aggressive, malignant neoplasm that has recently been characterized. It has not been associated with a primary visceral organ. In women, cases are even more rare and often have some ovarian involvement. CASE: An 11-year-old girl presented with abdominal pain, nausea, and vomiting. A CT scan revealed a large heterogeneous pelvic mass with cystic components and an 8-cm midabdominal mass. During exploratory laparotomy, the patient was found to have a pelvic mass measuring 12. 9 cm replacing normal ovarian tissue. The midabdominal mass was also removed. Pathology, cytology, and immunohistochemistry confirmed a desmoplastic small round cell tumor. Even with aggressive surgical and medical intervention, the patient died 11 months after initial diagnosis. CONCLUSION: We present a rare small cell tumor that is associated with ovarian involvement. The prognosis in these patients is extremely poor and very few survivals have been reported.     

Serologic, immunologic, and clinical features of parenteral drug users from contrasting populations

We screened inpatient and outpatient parenteral drug users with no clinical evidence of AIDS for immunodeficiency and antibodies to HTLV-III by ELISA. Among 20 outpatient drug users, 5 (25%) were seropositive. Three of these (and 2 who were seronegative) had low T-cell ratios. Over 6 months, 1 seropositive patient with a low ratio developed oral thrush and weight loss. We also studied 13 parenteral drug users hospitalized for conditions other than AIDS. Eight had low T-cell ratios, and at least 6 of these developed AIDS or ARC within 4 months. Serum from 8 of 13 inpatients was available for HTLV-III testing: 6/8 were seropositive and 3 of these 6 were among those developing AIDS or ARC. Abnormal T-cell ratios among all patients were associated with abnormal HTLV-III serology (p = .02). Of the 7 patients who developed AIDS or ARC, 4 were tested for both antibodies and T-cell ratios: all 4 were seropositive and had low ratios. A low ratio (p = .0004), a positive ELISA (p = .014), and abnormalities of both tests (p = .001) were associated with the development of AIDS or ARC. Of the 26 patients without AIDS or ARC, 3 were lost to follow-up and 23 did not develop AIDS or ARC. Six of these 26 had abnormal ratios. Of the 21 patients who did not develop AIDS or ARC and who were tested for HTLV antibodies, 2 were lost to follow-up. Seven of 21 were seropositive and 2/21 were both seropositive and had a low ratio. One of these 2 seropositive patients with low ratios also had lymphadenopathy, but he was lost to follow-up. The other had no adenopathy and remained well until her death from trauma a year later. This study found two populations with very different risks. Six of 13 hospitalized parenteral drug users and only 1 of 20 healthy outpatients developed AIDS or ARC.     

Complications associated with surgical treatment of congenital intrinsic duodenal obstruction

Although survival rates for infants undergoing surgical treatment for congenital intrinsic duodenal obstruction are high, long-term follow-up suggests a high complication rate related to surgical therapy. We reviewed 33 neonates who underwent surgery for congenital intrinsic duodenal obstruction during the past 10 years. There were 20 girls and 13 boys; the mean gestational age was 36 weeks, and mean birthweight was 2,485 g. Bilious vomiting and intestinal obstruction were the most frequent presenting symptoms. Hydramnios was present in 75% of cases and 21% had associated Down's syndrome. Findings at laparotomy included duodenal atresia (14), annular pancreas (11), and duodenal diaphragm (8). The most frequent surgical procedure was side-to-side duodenoduodenostomy (DD), followed by duodenojejunostomy and resection of web with Heineke-Mikulicz type duodenoplasty. Bowel transit was reestablished at a mean of 13.1 days (range, 6 to 45 days). Seventy-percent of patients developed postoperative complications, the most frequent being megaduodenum with blind loop syndrome or bile reflux gastritis (22%), cholestatic jaundice (17%), gastroesophageal reflux (17%), delayed transit (8%), and bowel obstruction (8%). Six patients (18%) required secondary surgical procedures for postoperative complications (ie, megaduodenum, nonfunctioning anastomosis, missed intrinsic stenosis). Two patients died (6%). Stagnation and functional obstruction in the proximal duodenum is the main factor influencing the morbidity rate among these patients. Consideration should be given to the tapering duodenoplasty and diamond-shaped anastomosis in order to help reduce problems associated with megaduodenum and help restore earlier bowel transit.     

Congenital esophageal stenosis: the differential diagnosis and management

Congenital esophageal stenosis (CES) is a rare congenital abnormality that is difficult to diagnose and often masquerades as other types of structural esophageal disease. We report three cases of CES with different presenting symptoms. We advocate for balloon dilation as the preferred first approach to therapeutic intervention. CES is an important clinical entity in the evaluation of pediatric esophageal disorders and should be suspected in young infants with dysphagia.     

Esophageal leiomyoma in children: two case reports and review of the literature

Leiomyoma of the esophagus is not uncommon in the adult population but is rarely seen in children; only 20 cases have been reported in the pediatric population. In this paper we describe two cases of esophageal leiomyoma in female patients aged 6 and 13 years and review previous reports. Several differences were noted between the pediatric and adult population. The mean age in children is 14 years (range, 4 to 20 years). Leiomyoma appears 1.71 times more often in females than in males. Localized lesions are found in only 9%, whereas the diffuse form predominates in 91%. The entire esophagus may be involved 35% of the time, and encroachment on the cardia or upper stomach occurs in 70%. Leiomyomas associated with familial syndromes (familial leiomyoma and Alport's syndrome) occur in 22% of the cases. Major symptoms include dysphagia (86%), dyspnea (36%), vomiting (27%), retrosternal pain (27%), and coughing (22%). The initial diagnosis following contrast studies is most often achalasia. The diagnosis of leiomyoma is made only with subsequent endoscopy. Enucleation was performed in only 11% of the cases; surgical resection (including part of the stomach) was necessary in 78% with a 21% postoperative mortality. Esophageal leiomyoma should be considered in the differential diagnosis of mediastinal masses and esophageal obstruction. Accurate preoperative diagnosis is desirable in order to plan proper surgical treatment.