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Biomedical subjects

M Emms

Publications and source records attributed to M Emms.

22 records · Page 2Linked to original sources

Childhood pulmonary blastoma: a pleuropulmonary variant of the adult-type pulmonary blastoma.

Two fatal childhood cases of the rare pulmonary blastoma are reported. One was associated with a congenital cystic adenomatoid malformation. Both neoplasms extended to involve visceral pleura and were entirely composed of blastemal and mesenchymal elements without recognizable neoplastic epithelial components. The mesenchymal component in both instances consisted of malignant rhabdomyoblasts, undifferentiated mesenchyme, and differentiated, apparently benign, cartilage. Review of the literature suggests that these features may be specific for the childhood forms of pulmonary blastoma. It is further suggested that pulmonary blastoma, malignant mesenchymoma of the lung, and primary pulmonary rhabdomyosarcoma may have a common pathogenetic origin.

Carcinoembryonic Antigen↗

Bilateral renal malakoplakia in infancy.

Two male infants aged 6 months presented with Escherichia coli septicaemia and urinary tract infection. Despite seemingly appropriate antibiotic therapy, a swinging fever, painful enlargement of both kidneys, sterile leukocyturia and renal failure persisted. Excretory urography, ultrasound scan, computerized tomography and magnetic resonance imaging showed diffuse infiltrative disease. 99mTc dimercaptosuccinic acid uptake was minimal, but a 67Ga-citrate scan showed striking diffuse uptake by both kidneys suggestive of inflammatory cell infiltration. Bilateral renal parenchymal malakoplakia was diagnosed on tissue examination. Bilateral parenchymal involvement of the kidneys by this chronic granulomatous disease has not previously been reported in infancy and is fatal if untreated. The lesion is believed infection-induced, due to defective bacterial activity of the macrophages, possibly related to an abnormally low cGMP/cAMP ratio. Treatment with intracellularly active trimethoprim-sulphamethoxazole, rifampicin and ascorbic acid resulted in complete recovery of the one infant so treated over a period of months.

Escherichia coli Infections↗

Malignant ectomesenchymoma in childhood.

Five childhood malignant ectomesenchymomas are reported from three centers in three countries. The children were all younger than 3 years (four less than 12 months), four were boys, and four tumors were sited in the pelvis or external genitalia. All tumors had distinctive light microscopic features of rhabdomyosarcoma and three also demonstrated small numbers of included neuronal cells. Immunohistochemical studies and transmission electron microscopy revealed the additional presence of neurogenic components in the remaining two patients. An additional epithelial component was found by immunohistochemistry in one tumor, which suggests a pluripotential origin that still requires karyotypic investigation. Aggressive chemotherapy and adequate surgical excision have thus far been effective in providing disease-free follow-up for periods of 7 to 50 months. It is implied that because the biological behavior and morphology closely resemble those of rhabdomyosarcoma and because the neurogenic component is often inconspicuous, many malignant ectomesenchymomas may be misdiagnosed as rhabdomyosarcomas.

Child, Preschool↗

Pluripotential melanoblastoma, a unifying concept on malignancies arising in congenital melanocytic nevi: report of two cases.

Congenital melanocytic nevi are benign lesions present at birth and considered to be caused by a maldevelopment of the neural crest. The malignant potential of the congenital melanocytic nevi have been extensively addressed by several authors, and malignant melanoma is the most frequent neoplasm arising in these lesions. The present report describes two patients with congenital melanocytic nevi in which malignant melanoma with undifferentiated areas showing rhabdomyoblastic differentiation developed. The findings suggest that these mixed neoplasms may be recapitulating the differentiation potential of the ectomesenchyme-neural crest cells. We advocate the term "melanoblastoma" when referring to them.

Child↗