[Plexiform fibrohistiocytic tumor. Histology, immunohistology and ultrastructure].

An example of a plexiform fibrohistiocytic tumour (PFT) is presented for the first time in the German-language literature. The diagnostic criteria for this rare soft-tissue neoplasm of childhood and young adulthood, which is mostly subcutaneous in location and has a tendency to recur, include the plexiform structure which gives it its name, extensive siderin deposits and numerous osteoclast-like giant cells. The development of the tumour described here, which occurred in the field of radiation of a malignant haemangiopericytoma excised 7 years previously, indicates the possibility of induction of PFT by radiation.

Clinico-pathological criteria with prognostic relevance in hepatoblastoma.

We investigated clinical data and histological specimens of 46 patients with a hepatoblastoma (HB) for prognostic criteria. Disease-free survival (DFS) of 23 patients treated in the German Cooperative Study HB-89 (1988-1990) was 83%, in contrast to 40% in 10 children with other chemotherapy regimes (1977-1987) and 38% in 13 with only a tumour resection (P = 0.005).

Treatment of malignant testicular tumors in childhood: results of the German National Study 1982-1992.

The German Cooperative Protocol for treatment of testicular germ cell tumors in childhood registered 106 patients from January 1982 through February 1992. Sixty-one patients suffered from yolk sac tumors (YST); 25 patients from differentiated teratomas (TD); 19 patients from malignant teratomas of either intermediate (MTI), undifferentiated (MTU), or trophoblastic type (MTT), and 1 patient from a seminoma. A stratified chemotherapy based on stage and histology was administered in addition to unilateral orchiectomy: Standard chemotherapy consisted of four treatments with vinblastine, bleomycin, and cisplatinum.

Intraabdominal desmoplastic small-cell tumor with divergent differentiation: clinicopathological findings and DNA ploidy.

Five cases of intraabdominal small-cell tumor with divergent differentiation are reported. All patients were of male sex. They were 10, 15, 20, 21, and 30 years of age at time of diagnosis, respectively.

DNA analysis in hepatoblastoma by flow and image cytometry.

Background: In several types of tumors, including hepatocellular carcinoma, prognosis could be correlated with DNA ploidy. Few studies have been performed on hepatoblastoma with contradictory results.

Methods: Twenty-nine cases of nonpretreated hepatoblastoma were studied with flow cytometry and image cytometry for DNA index and proliferation index using paraffin-embedded tissue.

Macrophage production of basic fibroblast growth factor in the fibroproliferative disorder of alveolar fibrosis after lung injury.

In organ repair following injury, macrophages accumulate and granulation tissue, comprised of fibroblasts and endothelial cells, develops in the injured area. Basic fibroblast growth factor (bFGF), a potent stimulator of fibroblast and endothelial cell growth, has been linked to the fibroproliferative process. Macrophages are thought to play a central role in the fibroproliferative response, and prior studies indicate that they produce bFGF.

Mutation of the p53 gene in neuroblastoma and its relationship with N-myc amplification.

Mutation of the p53 tumor suppressor gene frequently occurs in a variety of tumors including lung, breast, gastrointestinal, and brain, as well as lymphomas-leukemias. Neuroblastoma, one of the most common solid tumors in childhood, often has amplification of the N-myc gene. We examined for mutations of the p53 tumor suppressor gene by single-strand conformational polymorphism using polymerase chain reaction products and direct sequencing method in neuroblastoma; in addition, we assessed the relationship between p53 mutation and N-myc gene amplification in the disease.

Discrimination between immunoaccessory and phagocytic monocytes/macrophages of the skin in paraffin-embedded tissue by the monoclonal antibody Ki-M1P.

The variety of functions performed by monocytes and macrophages is reflected in their phenotypic diversity. Investigation of this complex system is facilitated by Ki-M1P, a new monoclonal antibody which recognizes a differentiation antigen on monocytes/macrophages in paraffin-embedded tissues. To test its usefulness as a pan-macrophage marker in the skin, we immunohistochemically analysed paraffin-embedded biopsy material from seven healthy individuals and 190 patients with a variety of dermatoses.

[Therapy of testicular germ cell tumors. Current status of the MAHO studies].

The GPOH-MAHO trials designed in 3/82 and 7/88 for treatment of childhood testicular germ cell tumors registered 3/92 105 pts. In MAHO 82 study 57 pts. and in MAHO 88 study 48 pts.

[Improved prognosis of intracranial germ cell tumors by intensified therapy: results of the MAKEI 89 therapy protocol].

Germ cell tumors of the central nervous system are histological identical to the extracranial tumor sites. According to the localisation germ cell tumors of the CNS are different in symptoms, diagnostic approaches, kind and location of metastases and stratification of therapy. Since 1986 patients with intracranial germ cell tumors are registered in the ongoing study for non-testicular germ cell tumors (MAKEI) of the German Society of Pediatric Oncology and Hematology, and are treated in accordance to therapy guidelines for extracranial sites.

Rhabdomyosarcomas with primary presentation in the skin.

Five primary and two metastatic rhabdomyosarcomas (RMS) with primary presentation in the skin were studied by conventional light microscopy and immunohistochemistry. These cases account for only 0.7% of the 682 cases of RMS collected at two large institutions with a main interest in soft tissue tumors.

Keratin-positive, epithelial membrane antigen-positive solitary pelvic tumor with ultrastructural features of large cell lymphoma.

A case of an unusual malignant solitary pelvic tumor is presented. The neoplastic cells were positive for keratin and epithelial membrane antigen. Although ultrastructural features (prominent nuclear pleomorphism, abundant polyribosomes, and absence of cell junctions) were those typically seen in large cell lymphomas, lymphoid markers were not detected, and immunoglobulin heavy-chain gene and T-cell receptor beta-chain gene rearrangements were not identified.

Spindle cell variants of embryonal rhabdomyosarcoma in the paratesticular region. A report of the Intergroup Rhabdomyosarcoma Study.

We reviewed 173 cases of paratesticular rhabdomyosarcoma (RMS) of Intergroup Rhabdomyosarcoma Studies (IRS)-I, -II, and -III for evaluation of possible histological factors that might account for the good prognosis of these patients. Almost all cases (161 of 173 cases, 93.1%) occurring in this site were of embryonal histology.

Mesoblastic nephroma metastatic to the lungs and heart--another face of this peculiar lesion: case report and review of the literature.

A case of mesoblastic nephroma in a 14-month-old girl who developed consecutive metastases in the lung and the heart is presented. This tumor is considered to be benign and cured by surgery only. Recurrent cases are extremely rare and usually related to unclear surgical margins.

Rare tumors in childhood: pathological aspects. Experience of the Kiel Pediatric Tumor Registry.

A wide variety of rare tumors can occur in childhood and adolescence. Tumors can be rare in any age group; common tumors of adults can appear in childhood and adolescence, and many rare tumors and tumor-like lesions show a predilection for younger age groups. Comparatively frequent tumors can present with rare histologic features or can occur in rare atypical locations, and, finally, seemingly common but, in fact, rare tumors have to be distinguished from truly common but rarely recognized tumors.

Infectious risks of Broviac catheters in children with neoplastic diseases: a matched pairs analysis.

In the present study the complication rate of Broviac catheters in the therapy of children with cancer was determined. Of special interest was the question of to what extent the incidence of bacteremias is increased by the implant. For this reason the method of matched pairs analysis was chosen comparing 55 patients with 61 catheters to 1 child each who received the therapy via peripheral veins.

[Experiences with surfactant therapy of adult respiratory distress syndrome].

We report on 2 patients with ARDS, who underwent a therapy with surfactant. In both cases the underlying reason for the lung disease probably was a viral pneumonia. In both patients the gas exchange improved after tracheal instillation of surfactant.

[Preoperative chemotherapy of nephroblastoma. Preliminary results of the SIOP-9/GPO therapy study].

The therapeutic strategy of the SIOP-9 study includes pre-operative chemotherapy for all patients with nephroblastoma diagnosed by imaging methods aged between 0.5 and 16 years. By pre-operative chemotherapy the rate of radical resectable tumors should be increased and thereby the intensity of postoperative therapy, in particular of radiotherapy, diminished.

Spindle cell rhabdomyosarcoma. A prognostically favorable variant of rhabdomyosarcoma.

Twenty-one cases of embryonal rhabdomyosarcoma, composed mainly of elongated spindle cells arranged in a fasciculated or storiform pattern, were retrieved from the files of the German-Italian Cooperative Soft Tissue Sarcoma Study. The term spindle cell rhabdomyosarcoma is proposed to designate this histotype. Spindle cell rhabdomyosarcoma predilected male patients (18 males, three females) and involved mostly the paratesticular area (12 cases) and the head and neck region (six cases).

Malignant renal tumors of childhood.

The prognosis in nephroblastoma (Wilms' tumor) has been improved considerably by treatment protocols combining surgery, chemotherapy, radiation therapy, and, in some clinical trials, pre-operative chemotherapy. Cure is now achieved in most patients. All clinical trials have employed treatment strategies tailored to the individual risk of the patient, including the histological subtype of the tumor.

Malignant rhabdoid tumor: primary intracranial manifestation in an adult.

Clinical, histopathological, and ultrastructural features of an extended intracranial malignant neoplasm are presented. Following incomplete extirpation of a large epidural tumor at the temporal base of the skull, the 21-year-old male patient suffered from several local recurrences and died at the age of 27. The neoplasm was defined by its histopathological and ultrastructural characteristics and by the pattern of its antigenicity as a malignant rhabdoid tumor (MRT).

Serum vanillylmandelic acid/homovanillic acid contributes to prognosis estimation in patients with localised but not with metastatic neuroblastoma.

In 211 patients with neuroblastoma, serum vanillylmandelic acid (VMA) and homovanillic acid (HVA) levels were determined and correlated to stage, histological differentiation, ferritin, neuron-specific enolase, lactate dehydrogenase (LDH) and outcome. Elevated serum VMA and/or HVA levels were found 16% less frequently than elevated urine levels. The incidence of the elevated serum levels increased with stage (stages I-III 58%, IV 78%, IVS 100%).

Malignant peripheral neuroectodermal tumor and its necessary distinction from Ewing's sarcoma. A report from the Kiel Pediatric Tumor Registry.

A new classification scheme is proposed for the differential diagnosis of Ewing's sarcoma and malignant peripheral neuroectodermal tumor (MPNT) based on conventional light microscopic and immunohistochemical findings. The presence of Homer-Wright rosettes and/or the expression of at least two neural markers is diagnostic of MPNT Ewing's sarcoma. Ewing's sarcoma was diagnosed in cases lacking Homer-Wright rosettes and expressing no neural marker or only one in immunohistochemistry.

[Cystic space occupying lesion of the upper pole of the kidney in a newborn infant. Case report and differential diagnosis].

In a newborn female infant a cystic lesion in the upper pole of the right kidney was detected by sonography 4 weeks before an uneventful delivery. In the postnatal period the sonographic examination, IVP, CT, and the biochemical data as well were not able to classify definitely this lesion as a benign cyst, adrenal hemorrhage or cystic malignoma. Therefore a laparatomy was performed, which revealed a cystic neuroblastoma.

Infantile desmoid-type fibromatosis. Morphological features correlate with biological behaviour.

It is generally agreed that the biological behaviour of infantile desmoid-type fibromatosis is unpredictable in terms of the likelihood of local recurrence. We evaluated the prognostic importance of the number of slit-like blood vessels with a mean diameter of less than 20 microns and of the quantity of undifferentiated mesenchymal cells. In addition, we studied the immunophenotypic characteristics of the proliferating cells and their nucleolar organizer region (AgNOR) numbers and distribution.