Peter E. Campbell

Comparison of PCR‐ELISA and galactomannan detection for the diagnosis of invasive aspergillosis

Aim: To compare PCR with galactomannan antigen detection for the diagnosis of invasive aspergillosis (IA). Methods: We prospectively collected serial blood samples from haematological patients at risk of IA, and analysed their samples retrospectively for galactomannan (GM) antigen using the Platelia test and for aspergillus DNA using an in‐house PCR‐ELISA assay. Matched GM and PCR analyses were performed on 263 samples from 25 patients. Patients were classified for potential IA according to international consensus criteria, with five patients classified as positive (four proven, one probable) and 20 classified as negative (seven possible, 13 no evidence IA). Results: All five patients with IA were positive by PCR with positive results in 24 of 82 samples, whereas three of five patients were positive by GM with four of 82 samples being positive. Three of 20 patients without IA were positive by PCR in 18 of 181 samples, whereas corresponding results for GM detection were one of 20 and one of 181, respectively. Adjustment of ELISA cut‐off values and/or the requirement for two consecutive samples to be positive generated different results; however, lowering the positivity index (PI) for GM detection to 0.5 did not improve the sensitivity of the assay. Optimal results for PCR detection and GM were: 100% and 60% sensitivity, 85% and 95% specificity, 0.625 and 0.75 positive predictive value, and 1.0 and 0.8 negative predictive value, with a false‐positive sample rate of 8 and 0.4%, positive likelihood ratio of 6.66 and 11.99 and negative likelihood ratio of 0 and 0.42, respectively. Conclusions: This PCR method is very sensitive for the diagnosis of IA but is associated with a moderate rate of false positives; the GM assay exhibited poor sensitivity but high specificity. Further evaluation of PCR assays for the diagnosis of IA and other invasive fungal infections is warranted.

Sweat gland carcinomas in children

The clinicopathologic features of sweat gland carcinomas in three girls and one boy, aged 7 months to 10 years, are presented. The histologic and ultrastructural features suggested malignant clear cell acrospiromas. Rapid extensive metastases developed in two and early local recurrence in one. No obvious effect of chemotherapy was noted in disseminated disease. Wide local excision is advised because of the risk of local lymphatic infiltration. Cancer 53:1222‐1227, 1984.

Primitive neuroectodermal tumour of the central nervous system associated with malignant rhabdoid tumour of the kidney: report of a case

Malignant rhabdoid tumour of the kidney is a recently reported tumour presenting in young children. Irrespective of stage and despite intensive chemotherapy these tumours have a poor prognosis, with death usually occurring within a matter of months. A recent report has shown the association of second embryonal tumours of the central nervous system occurring in patients with the renal tumour; most of these second tumours have occurred in the posterior fossa. We report here an infant who presented with a mass in the right groin, showing features of a poorly differentiated sarcoma, possibly rhabdomyosarcoma. Further investigations revealed a tumour in the lower pole of the right kidney which was subsequently shown to be a malignant rhabdoid tumour. The child was given chemotherapy but re‐presented at 10 months of age with hydrocephalus, irritability and spasms leading to death. At autopsy a large tumour was found filling the right lateral and third ventricles; histology showed a primitive neuroectodermal tumour with focal astrocytic differentiation. Residual rhabdoid tumour was restricted to a few para‐aortic lymph nodes and focal lymphatic micrometastases in lungs. The association of two embryonal neoplasms of possible similar histogenesis is discussed.

Malignant carcinoid tumors in children

The clinical, light microscopic, and ultrastructural features of four malignant carcinoid tumors in children, three boys and one girl ages 8–14 years, are described. Extensive metastases to multiple organs were present in three, and in the fourth child there was diffuse local infiltration of the bowel wall, which resembled a lymphoma. The primary tumor arose in the ileum in one child and in the transverse colon in another. In two children, the primary sites could not be determined; one patient is still alive and in the other, permission for autopsy was refused. Electron microscopy showed moderate numbers of neurosecretory granules in some cells in all cases. One patient with extensive metastases showed repeated partial response to radiotherapy and chemotherapy. Eight benign appendiceal carcinoids were seen at the same hospital over the same period, suggesting malignant carcinoids may be more common in children than often assumed.

Pulmonary complications of rubella embryopathy

The clinical and pathologic features of 6 infants with rubella embryopathy who died from the pulmonary complications of the disease are reported together with details of a seventh patient who has apparently recovered. The 7 patients presented with symptoms of cough and breathlessness between 1 and 6 months of age and had clinical and radiologic features of an interstitial pneumonitis. The lungs from the patients who died showed a spectrum of pathologic change from an acute generalized pneumonia with hyaline membrane formation to chronic interstitial pneumonitis; one had secondary infection with Pneumocystis carinii .

Angiomatosis: A vascular malformation of infancy and childhood. Report of 17 cases

&NA; Angiomatosis is a complex vascular malformation of infancy and childhood consisting of proliferating blood vessels with accompanying mature fat and fibrous tissue, lymphatics and sometimes nerves, that may involve skin, subcutaneous tissue, skeletal muscle and occasionally bone; lesions are non‐encapsulated with poorly defined infiltrative borders. Treatment is surgical, with local recurrence being common. We report 17 cases of angiomatosis presenting in children. Recurrences occurred in 10 patients, with multiple recurrences occurring in four. One child was treated with foot amputation followed two years later by mid‐thigh amputation in an attempt to control local disease. Histology in all cases showed a mixture of small and medium‐sized blood vessels, fat, connective tissue and lymphatics; nerves were increased in several cases. All lesions showed nests of proliferating capillaries, arranged in a lobular pattern, pushing into adjacent muscle and fat. This appearance was not seen in a large comparison group of vascular soft tissue lesions, and may serve as an indicator of angiomatosis with its associated risk of recurrence.

Extrahepatic biliary atresia: Comments on the frequency of potentially operable cases

In a 10 year period, 35 infants with extrahepatic biliary atresia were encountered. Twenty-eight babies, who were subjected to surgery and operative liver biopsy, are discussed. Although an operable lesion was detected at laparotomy in only one baby, 5 further potentially correctable lesions were discovered in the 14 necropsies performed. Review of the literature revealed that few authors have discussed necropsy findings and that confirmation of the diagnosis by liver biopsy has been neglected in many cases, including some of those claimed as surgical cures. It is concluded that the frequency of potentially operable forms of biliary atresia is not really known.

Bilateral metachronous periosteal osteosarcoma

The first case of bilateral metachronous periosteal osteosarcoma (OS) is reported. A 14‐year‐old white boy presented with a 1‐month history of pain and swelling in his right thigh. Periosteal OS was diagnosed on a basis of the radiologic and pathologic findings. Treatment was with local resection and total hip replacement after a short course of high‐dose methotrexate; multi‐agent chemotherapy was continued postoperatively for 3 months. He remained well for 3 years. He then represented with a mass in the left femur that had been slowly growing for about 1 year. Radiologic and biopsy studies showed periosteal OS. Full investigations showed no evidence of metastatic disease. Treatment consisted of local resection without chemotherapy. He remained well for 6 months after the second excision until developing multiple pulmonary metastases. All further therapy was refused. The question as to whether the second tumor was a new primary lesion or a metastasis is discussed, together with possible differential diagnoses. Cancer 58:1139‐1143, 1986.

Thoracic neural crest tumors: A clinical review

The experience of a regional childrens hospital in the management of intrathoracic neural crest tumors over a period of 20 years is presented. Of 145 children with neuroblastoma between 1961 and 1980 inclusive, 115 had primary abdominal neuroblastoma (including pelvic) with a mortality of 86%; 30 patients had a thoracic neuroblastoma with a mortality of 16.5% (five patients). The more favorable outlook in this group should imply a more cautious approach to the initiation of treatment regimens which have their own morbidity and mortality.

Crohn's disease : the Melbourne experience

During the years 1979–1988, 63 children (38 male: 25 female) were first diagnosed as having Crohns disease on the basis of histological findings. Twenty-nine per cent were less than 10 years old at the onset of symptoms; 40% of these cases took more than 1 year to diagnose. Most patients (59%) presented with abdominal pain and diarrhoea, but 40% presented with: a non-specific illness (14%); upper gastro-intestinal tract symptoms (14%); growth failure (6%); systemic disease (5%), or peri-anal disease (1%). There were signs of abdominal tenderness, distension, or an abdominal mass in over 50% of cases. Peri-anal Crohns disease was present in 41% and a height less than the 10th centile was recorded at diagnosis in 45%. Children were treated with a combination of prednisolone and sulphasalazine. In 16 cases a bowel resection was performed. Surgery was indicated for localised stricture formation causing obstruction and for persistence of severe disease poorly responsive to steroids, particularly in the prepubertal child with growth failure. The extent and distribution of disease influenced outcome. Twenty-four of 39 children followed for more than 3 years are either well-controlled or disease-free at present.