Investigation of stillbirth.

The pathologic investigation of stillbirth is often frustrating, and may be viewed as unrewarding, because the reason for death of the infant frequently is not identifiable. This outline attempts to identify an approach to the examination of the stillborn and accompanying placenta, with appropriate use of ancillary studies that may uncover the cause of death and/or identify factors that could prejudice future pregnancies.

Local staging of Wilms' tumor--computerized tomography correlation with histological findings.

PURPOSE: The aim of this study was to assess the accuracy of computed tomography (CT) in the local staging of Wilms' tumor. METHODS: Twenty-six patients with unilateral, nonmetastatic Wilms' tumors were accrued over the period of 1990 through 1997. Preoperative CT staging was performed before surgical biopsy or excision. The abdominal CT images were reviewed and staged for local disease according to National Wilms' Tumour Study (NWTS) Group Staging V as either intrarenal disease (Stage I), local extrarenal extension amenable to complete local excision (Stage II), or advanced local disease (Stage III). Patients with metastatic (Stage IV) and bilateral disease (Stage V) were excluded from our study. Results were compared to histological findings. RESULTS: CT scan correctly staged 10 of 26 cases (38%) of Wilms' tumor. CT scan overstaged 12 of 16 (75%) localized renal disease (Stage I) and local extension (Stage II) tumors, and understaged 4 of 10 (40%) localized spread (Stage III) tumors. CONCLUSIONS: CT scan appears to have poor correlation to histological staging. Therefore, basing therapy solely on radiological imaging may lead to some patients receiving more intense therapy than necessary, whereas others do not receive sufficient therapeutic regimens. Histological assessment of the excised mass should remain the standard for staging Wilms' tumors.

Breast cancer in a 6-year-old child.

A 6-year-old girl presented for a second opinion with a 1-year history of an enlarging soft tissue mass just lateral to the right areola. She had been seen by a pediatric surgeon elsewhere who reassured the parents that the lesion was benign. Ultrasound scan showed a 1.5- x 1.5-cm cystic structure adjacent to the right breast bud. Excisional biopsy results showed secretory ductal adenocarcinoma. Modified radical mastectomy with axillary node dissection was performed. All 11 nodes were negative for metastatic disease. She is now disease free 3 years after diagnosis. Estrogen-progesterone receptors were negative, as was screening for BCR 1 and 2. This is the first report of cytogenetics showing an abnormal cell line with a reciprocal translocation between 12p and 15q. Although breast cancer is extremely rare in children, a history of a painless, enlarging, firm breast mass should raise concern about possible neoplastic disease. Cystic appearance on ultrasound scan caused by the pseudocapsule around the tumor may be a marker for secretory carcinoma. Histological evaluation of all suspicious masses should be obtained. Because of the risk of local recurrence and axillary metastases, the authors recommend modified radical mastectomy with axillary node dissection for children with secretory carcinoma of the breast.

Neutropenic enteropathy: a 10-year review.

PURPOSE: With the advent of more aggressive chemotherapy, the incidence of neutropenic enteropathy is increasing. This review was performed to (1) determine which children are affected, (2) identify predisposing factors, and (3) assess efficacy of treatment. METHODS: A 10-year (1988 to 1997) review identified 33 children who had 38 episodes of neutropenic enteropathy. Each presented with fever, abdominal pain, and chemotherapy-induced neutropenia. All were treated with fluid resuscitation, bowel rest, and broad-spectrum antibiotics. Surgical intervention was reserved for children with bowel perforation. RESULTS: Neutropenic enteropathy occurred in 24 children with hematologic malignancies and nine with solid tumors. This disease developed in three children after bone marrow transplantation for leukemia. All five patients in whom recurrent episodes developed were receiving chemotherapy for Burkitt's lymphoma. Cytosine arabinoside and VP16 were the most commonly implicated chemotherapeutic agents. Four children required laparotomy and right hemicolectomy, and all survived. Overall survival was 94%. CONCLUSIONS: Although previously described in children receiving chemotherapy for hematologic malignancies, neutropenic enteropathy also occurs in children who have solid tumors and after bone marrow transplantation. The use of cytosine arabinoside and VP16 may predispose patients to this disease. Children receiving chemotherapy for Burkitt's lymphoma are at increased risk for recurrent episodes. Excellent survival rate was attained with supportive care being used as the primary treatment modality.

Fibromatoses: clinical and pathological features suggestive of recurrence.

BACKGROUND/PURPOSE: Fibromatoses represent a spectrum of nonneoplastic spindle cell tumors frequently identified in childhood. Although they may be locally aggressive, they do not metastasize. The authors have reviewed their experience of these lesions in an attempt to identify clinical and pathological features suggestive of recurrence. METHODS: Clinical records and pathological specimens from 55 cases of fibromatosis diagnosed and treated at British Columbia's Children's Hospital from 1982 to 1995 were reviewed. RESULTS: Thirty boys and 25 girls with ages ranging from 1 month to 14 years were identified. Eighteen tumors (33%) were congenital. Diagnoses were as follows: musculoaponeurotic fibromatosis (n = 27), infantile myofibromatosis (n = 10), Dupuytren-type fibromatosis (n = 7), fibromatosis colli (n = 7), and digital fibroma (n = 4). Clinical follow-up showed a survival rate of 98%. The single mortality occurred in an infant born with an intraabdominal visceral myofibromatosis, which proved unresectable. Fifteen cases recurred (27%), and nine cases (16%) of infantile myofibromatosis spontaneously regressed. All remaining cases were controlled successfully by surgical resection. CONCLUSIONS: Clinical features suggestive of recurrence included (1) presentation at greater than 5 years of age, (2) extremity location of disease, and (3) incomplete surgical resection. Pathological features suggestive of recurrence were (1) microscopic evidence of tumor at resection margins, (2) mitotic index of 5 or more per 10 high-power fields, and (3) areas of necrosis and inflammation within the tumor.

Lipoblastoma and liposarcoma in children: an analysis of 9 cases and a review of the literature.

OBJECTIVES: To review the experience at a children's hospital of lipoblastoma and liposarcoma and to identify any factors that would differentiate one type of tumour from the other. DESIGN: A retrospective case series. SETTING: British Columbia's Children's Hospital a tertiary-care pediatric centre. PATIENTS: All patients with a pathological diagnosis of lipoblastoma and liposarcoma recorded over 12 years. MAIN OUTCOME MEASURES: The frequency of lipoblastoma and liposarcoma, identified from biopsy specimens of pediatric adipose tumours. The clinical, pathological and cytogenetic variables between lipoblastoma and liposarcoma. RESULTS: One hundred and forty-nine adipose tumours were recorded. Seven (4.7%) were lipoblastomas and 2 (1.3%) were liposarcomas. All tumours presented as asymptomatic, slow-growing, soft-tissue masses. The children with lipoblastoma tended to be younger, but 29% were over 3 years of age. The liposarcoma patients were aged 9 and 14 years. One liposarcoma was of myxoid type and the other was a round cell variant. Karyotypes were reported for 1 lipoblastoma and 1 liposarcoma. The myxoid liposarcoma karyotype was 46,XY,t(12;16)(q13;p11), and the lipoblastoma was reported as 46,XY,der(8)?t(8q;?),+mar. CONCLUSIONS: Lipoblastoma is an unusual childhood neoplasm and liposarcoma is very rare in children. Both tumours may present in a similar fashion, and differentiating them histologically can be difficult. Age cannot be relied upon to accurately predict their behaviour. The tumour karyotype is very helpful in differentiating these neoplasms.

Proximity injury by the ultrasonically activated scalpel during dissection.

The ultrasonically activated scalpel is a high-frequency oscillating instrument that is reported to have a decreased dispersion of energy to surrounding tissues during use. To determine if this effect is beneficial and safe to surrounding tissue, it was used on anesthetized adolescent swine to dissect the portal vein from the pancreas, the renal artery and vein from the renal hilum, the ureter from the retroperitoneum, the aorta from the inferior vena cava and the common bile duct from surrounding tissue. Three-second contact to intestine and nerve roots was also performed. Wedge biopsy specimens of liver and spleen were performed. Dissection technique used was as described by the company. Structures were dissected with electrocautery using similar techniques for comparison. Tissues were harvested and placed in formalin for histological analysis. Dissection with the ultrasonically activated scalpel was simple, achieved excellent hemostasis, and did not appear to damage adjacent tissue. Microscopic analysis showed adventicial and media injury to vascular structures. The ureter and common bile duct demonstrated marked injury with regions of transmural coagulation. Nerve and small bowel did not appear to have much injury from the 3-second contact with the instrument. This study indicated that although the ultrasonically activated scalpel can ease dissection with good hemostasis, care must be taken to avoid injury to adjacent structures. Although its lateral energy dispersion may be less than that of cautery, it can still cause transmural necrosis to major structures.

Tumor karyotype differentiates lipoblastoma from liposarcoma.

Lipoblastoma is a rare benign pediatric soft tissue tumor that may be difficult to distinguish from a myxoid liposarcoma clinically or histologically. The authors present a case of a progressively growing, locally invasive soft tissue tumor in a child. Tissue culture for cytogenetic study showed a breakpoint in the long arm of chromosome 8. A review of the literature showed seven case reports of lipoblastoma karyotype, six of which had a similar breakpoint in chromosome 8. This distinguishes it from the histologically alike myxoid liposarcoma, the karyotype of which typically contains the clonal anomaly t(12;16)(q13:p11). The authors recommend that when performing a biopsy of a childhood adipose tumor with unusual features, such as progressive or invasive growth, that fresh tissue be submitted for cell culture. The tumor karyotype will, in most cases, aid in differentiating lipoblastoma from myxoid liposarcoma.

Inguinal herniation with glial implants: possible complication of ventriculoperitoneal shunting.

The standard treatment of hydrocephalus is the insertion of a valve-regulated ventriculoperitoneal (VP) shunt, which may result in the development or clinical worsening of an inguinal hernia or hydrocele. A review of the British Columbia's Children's Hospital experience with VP shunt insertion (1983-1994) identified 304 patients who underwent VP shunt placement, 31 of whom subsequently required herniorrhaphy, 5 suffering recurrences. Two cases exhibited areas of glial differentiation (diffusely scattered in one sac from a bilateral repair, focally present in the second unilateral hernia repair) displaying cytoplasmic staining with glial fibrillary acidic protein and S100. At time of surgical repair of case 1 (bilateral hernia repair), the tip of the VP shunt was detected within the hernia sac exhibiting glial differentiation; no glial tissue was identified in the sac from the other side. We conclude that inguinal herniation is a common complication of VP shunt insertion, and the identification of glial tissue within such an inguinal hernia is a rarer complication, possibly occurring when the shunt tip lies in close proximity to the hernial mesothelial tissue.

Multiple entero-entero fistulae: an unusual complication of Henoch-Schönlein purpura.

Henoch-Schönlein purpura (HSP) is an immunologically mediated systemic vasculitis of small blood vessels that frequently involves the gastrointestinal tract. Surgeons often are asked to assess patients with HSP for abdominal pain. Common complications that lead to surgical intervention include intussusception, perforation, necrosis, and massive gastrointestinal bleeding. However, the development of multiple entero-entero fistulae has not been described previously. Herein the authors describe the case of a 10-year-old girl with HSP who had a typical rash, seizures secondary to central nervous vasculitis, and gastrointestinal involvement. The abdominal pain persisted for several days until marked fever and hypotension developed, which necessitated surgical intervention. During laparotomy, multiple entero-entero fistulae of the ileum were found, which required resection and primary anastomosis. Histological examination of the ileum was consistent with HSP vasculitis. The patient made an unremarkable recovery and has had no recurrence of symptoms.

Necrotizing fasciitis in childhood.

Necrotizing fasciitis is a rare entity in the pediatric population. Five cases of this soft tissue infection were treated at the authors' institution between January and December 1993. Three of the children were profoundly neutropenic secondary to chemotherapy. All five were treated with aggressive surgical debridement, frequent dressing changes, broad-spectrum antibiotics, and nutritional support. In addition, the patients with neutropenia received a combination of granulocyte-colony stimulating factor and granulocyte transfusions. One child died of overwhelming sepsis and bone marrow graft failure. The others eventually made a complete recovery. Necrotizing fasciitis may be becoming a more common problem in children. Aggressive chemotherapeutic regimens and more frequent use of bone marrow transplantation could be a factor in this. Early diagnosis and aggressive surgical therapy is critical. However, mortality may be significant, especially in patients with neutropenia. Leukocyte response to the infection may be a prognostic marker. Pseudomonas and enteric gram-negative organisms are seen frequently in immunocompromised children with necrotizing fasciitis. Antimicrobial selection should supply adequate coverage of these organisms.

Pilomatrixoma: a common, benign childhood skin tumour.

OBJECTIVE: To review recent experience with pilomatrixoma, particularly with respect to diagnosis. DESIGN: Chart review for an 8-year period. SETTING: A university-affiliated children's hospital. PATIENTS: Fifty children with 51 histologically proven pilomatrixomas. INTERVENTION: Excision of the lesion. MAIN OUTCOME MEASURES: Clinical presentation, diagnosis and pathological findings. RESULTS: The clinical presentation was typically that of an asymptomatic superficial mass that increased slowly in size and was located in the head and neck (78%) or chest and upper limbs. In one case the mass increased rapidly over 2 weeks and changed in colour from red to purple with areas of pink and tan. The referring diagnosis was incorrect in 94% of cases, and the preoperative diagnosis was incorrect in 57%. Pathologically the tumours were situated in the dermis or subcutaneous tissue. None were cystic, but several had areas of calcification. None of the tumours recurred after excision, but two patients had multiple lesions at different locations and times. CONCLUSIONS: Pilomatrixomas in children are simple to treat successfully but have variable clinical presentation. Early diagnosis is important so that unnecessary, aggressive surgery can be avoided.

In utero decompression of umbilical cord angiomyxoma followed by vaginal delivery.

An enlarging cystic-solid cord abnormality was found at prenatal identification to be an angiomyxoma. Partial in utero decompression of the 16 x 12 x 5 cm cystic component allowed uncomplicated spontaneous vaginal delivery at 36 weeks' gestation. Doppler studies had shown variable systolic/diastolic ratios in the solid component of the angiomyxoma.

Obstructive lung disease in children after allogeneic bone marrow transplantation.

Obstructive lung disease (OLD) has been described as a significant complication after allogeneic bone marrow transplantation (BMT). The incidence of OLD in adults appears to be low (approximately 3%), but there is little data for children. We analyzed 89 consecutive pediatric allogeneic BMTs, > or = 1.5 years post-BMT, performed at British Columbia's Children's Hospital from 1980 to 1992 for evidence of OLD. Diagnosis of OLD was based on clinical findings (nonproductive cough, wheezing, and dyspnea with no evidence of infection), pulmonary function tests (FEV1 < 80% and FEF25-75% < 60% predicted), lung biopsy, and computed tomography scan. Sixty-seven of the 89 children evaluated survived > or = 90 days and were classified as at risk for OLD. Thirteen of 67 (19.4%), developed OLD, 3 of which were transient. The development of OLD was strongly associated with the following high-risk groups: chronic graft-versus-host disease (GVHD) (37.1% OLD), increased donor age, acute GVHD, and either mismatched related or matched unrelated donor transplants. No correlation was found with methotrexate prophylaxis for GVHD, total body irradiation, or cytomegalovirus reactivity in either donor or recipient and the development of OLD. Further analysis of only children with chronic GVHD showed that liver involvement by GVHD before the onset of OLD (57.9%) was the only other significant predictive factor. We observed an overall increased prevalence of OLD in children compared with that previously reported in adults. Further studies are required to confirm whether age is a risk factor for development of OLD after allogeneic BMT.

Clinical variability within Brachmann-de Lange syndrome: a proposed classification system.

Seven patients, including two sibs, with the Brachmann-de Lange syndrome (BDLS) are presented as representative of the different types of BDLS in a proposed classification system. Type I ("classic") patients have the characteristic facial and skeletal changes of BDLS using the criteria in the diagnostic index of Preus and Rex. Type I is distinguished from the other subtypes by prenatal growth deficiency (< 2.5 S.D. below mean for gestation) becoming more severe postnatally (< 3.5 S.D. below the mean), moderate to profound psychomotor retardation, and major malformations which result in severe disability or death. Type II ("mild") BDLS patients have similar facial and minor skeletal abnormalities to those seen in type I; however, these changes may develop with time or may be partially expressed. Patients with type II BDLS are distinguished from those with other types by mild to borderline psychomotor retardation, less severe pre- and postnatal growth deficiency, and the absence of (or loss severe) major malformations. Behavioral problems can be a significant clinical problem in type II BDLS. Type III ("phenocopies") BDLS includes patients who have phenotypic manifestations of BDLS which are causally related to chromosomal aneuploidies or teratogenic exposures.

Malignant fibrous histiocytoma in childhood.

BACKGROUND: Malignant fibrous histiocytoma (MFH) is a neoplasm of late adult life and often is reported in the pediatric population. It is thought to behave more benignly in children than in adults. METHOD: Clinical and pathologic features, treatment, and outcome of nine pediatric patients seen at British Columbia's Children's Hospital between 1983 and 1990 were examined. The literature regarding pediatric malignant fibrous histiocytoma was reviewed. RESULTS: The tumors included one primary renal tumor and two occurring in the orbit after radiation therapy for retinoblastoma during the neonatal period. Histologic examination showed that six tumors had a storiform-pleomorphic pattern, one was myxoid, and two were angiomatoid in type. Six children are alive with a disease-free survival of 20 months to 8 years after surgical resection. Two of these received adjuvant chemotherapy, none had radiation therapy. Three patients have died of disease, two with pulmonary metastases that developed despite multiagent chemotherapy and radiation therapy. Poor outcome was associated with large tumors, deep and proximal location, and the storiform-pleomorphic histologic type with atypical mitoses. CONCLUSION: Malignant fibrous histiocytoma is similar in children and adults. Surgery is the mainstay of therapy, but the risk of local recurrence and pulmonary metastases indicates the need for adjuvant therapy in selected patients.

Adenocarcinoma in childhood Barrett's esophagus: case documentation and the need for surveillance in children.

A 17-yr-old boy underwent esophagectomy for multifocal high-grade dysplasia and adenocarcinoma complicating Barrett's esophagus (BE). He is believed to be the first child or young adult to have prolonged healthy survival following resection of esophageal adenocarcinoma. Dysplasia in a short retained segment of his Barrett's mucosa appears to have regressed with acid-suppressing therapy. Of nine other reported cases of adenocarcinoma in young people 11-25 yr of age, all died. All had progressive dysphagia and an esophageal mass at presentation, unlike our patient who had only histologic evidence of cancer at presentation. This was found only after repeated and extensive biopsy of the esophagus. We conclude that adenocarcinoma does occur under age 25 yr as a complication of BE arising in childhood, and it may be curable if diagnosed early. Endoscopic surveillance with multiple stepwise biopsies, beginning at age 10 yr, is suggested in those few children who have BE with specialized mucosa and goblet cells.

Rhabdomyosarcoma arising within congenital pulmonary cysts: report of three cases.

Over the past 9 months, three cases of primary pulmonary rhabdomyosarcoma have been treated at British Columbia Children's Hospital. Two patients (aged 24 and 37 months) presented with spontaneous pneumothoraces and had cystic changes in the affected lung on chest radiograph. The third patient (aged 42 months) was evaluated for chronic cough, fever, and failure to thrive. Chest x-ray showed a large mass in the left lower lobe as well as mediastinal adenopathy. All three of these lesions originated within congenital lung cysts, one a peripheral bronchogenic cyst and the others cystic adenomatoid malformations. This report suggests that there is a significant risk for the development of rhabdomyosarcoma within malformed pulmonary tissue.