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[Morphology of neuroblastoma. Light and electron microscopic studies as a contribution on diagnosis and differential diagnosis].

The neuroblastoma is one of the most frequent malignant solid tumors in childhood and is thus of great practical importance. The origin of neuroblastoma cells from neural crest derivatives is generally accepted now, and this histogenesis explains some biochemical and morphological characteristics of the tumor. The cytological and histological features of neuroblastomas can be rather varying and, therefore, the diagnosis and differential diagnosis may be difficult. Our study presents the findings of 48 neuroblastomas after light microscopic examination and the ultrastructural characteristics of 8 neuroblastomas and 1 ganglioneuroma. At light microscopic level, completely undifferentiated neuroblastomas and tumors with variable degrees of differentiation were identified. The differentiation of the tumor tissue to ganglion cell-like elements was indicated by an increasing amount of cellular cytoplasm with development of a cytoplasmic process as well as an alteration of the picture of the nucleus (nuclear enlargement and a clearly visible nucleolus). Differentiation to Schwann cell-like elements was occasionally observed, too. Electron microscopically, in all tumors neurosecretory granules could be recognized, and in the better differentiated areas neurite-like cytoplasmic projections were detectable. Thus, the electron microscopy can be a valuable aid in establishing an unequivocal diagnosis. The histology of neuroblastomas is said to be of prognostic significance. Therefore, grading schemes of malignancy were developed. At present, the grading procedure after Hughes and coworkers is mostly used. The criteria of this grading system are presented and interpreted. Finally, those tumors are briefly characterized which play the main role in the differential diagnosis, i.e. juvenile rhabdomyosarcomas, Ewing's sarcoma (including the extraskeletal type), lymphoblastic lymphoma and histiocytic reticulosarcoma. The most important clinicopathologic differences in comparison to neuroblastomas are referred to. Using a large scale of morphologic methods as well as considering clinical and paraclinical parameters, the exact diagnosis of neuroblastoma should be possible in nearly every case.

Adolescent↗

[Reticulosarcoma of the stomach. Apropos of 3 cases].

The authors report 3 cases of gastric reticulum cell sarcoma upon 58 tumours of stomach observed in the period 1975-1978. They examine the anatomopathological aspects in conformity with the last classification, they dispute about the clinical aspects and they accentuate the difficulty whether clinical or instrumental to succeed in an early diagnosis but they confirm also the opinion a surgical therapy extremely aggressive in sole possible. This therapy aggressive, eventually associated with the irradiation therapy and sometimes with the chemotherapy, makes the prognosis of types of tumours remarkably better than forms carcinomatous.

Antineoplastic Agents↗

[Radiation therapy and chemotherapy of primary malignant tumors of the bone (author's transl)].

In recent years, cytostatic chemotherapy has been added to the treatment of primary malignant bone tumors. Long-term results are not yet available. Since many of these tumors metastasize through the blood stream, the prognosis has been poor. Now a general improvement in the total number of cures is expected. In osteogenic sarcoma, amputation or exarticulation of the involved extremities is most frequently suggested; one can, however, continue to use preoperative radiotherapy. After 3 or at the most 6 months, amputation is carried out if the patient is free of metastases. Adjuvant chemotherapy should be carried out in every case. The side effects in the irradiated area, however, are considerably increased and, as a result, immediate amputation will become more and more popular. Irradiation of the primary tumor plays a decisive role in Ewing's sarcoma, surgical removal of the tumor does not require radicality at all costs. In reticulosarcoma of the bone, surgical intervention is limited to a biopsy because of high radiosensitivity. Adjuvant chemotherapy should be carried out in every case since a generalization is to be expected in 30% of the patients. The indications for "prophylactic" radiotherapy of the lung and the neurocranium were discussed.

Adolescent↗

[Radiation therapy and treatment results in carcinoma of the esophagus (author's transl)].

Results from radiotherapeutic treatment of 200 patients with malignant tumors of the esophagus are reported. The principal part is represented by squamous cell carcinomas with 88%, followed by adenocarcinomas with 4.5%, by more or less differentiated solid carcinomas with 4%, and small cell carcinomas with 1.5%. Sarcoma was found in three cases, one of them a myosarcoma, another a reticulosarcoma. An ectopic carcinoma of the gastric glands was present in one case. The staging was performed according to the TNM system. A focal dose of 6000 to 6500 rd within 6 or 8 weeks was tried for, using 60Co-gamma-rays. The one-year and five-year survival rates in stage T2N0M0 amounted to 80% and to 17%; no survival was obtained in stage T3N0M0. Out of the total of stage T2-3N0M0 cases 24% obtained one-year survival, and 2% five-year survival. In stages T3N2-3M0 and T3Nx-3M1 after one year 9% and 7% of the patients were alive. The mean survival time of stage T2N0M0 cases was 30.5 months; of T3N0M0, 7.3 months; of T3N1-3M0, 5.2 months; of T3Nx-3M1, 3.8 months. The one-year and five-year survival rates, being related to the irradiation dose administered, amounted to 28% and to 2% after a minimum dose of 5000 rd to the focus. With less than 5000 rd, none of the patients survived two years. The bad results of treatment demand new therapeutic pathways. Short-term pre-irradiation followed by surgical treatment is discussed as well as a combined radiation therapy with cobalt-60 and neutrons or a combined radio-chemotherapy using cytostatics.

Adenocarcinoma↗

[Chemoradiation pathomorphosis of Ewing's tumor and reticulosarcoma].

The paper is concerned with the results of a morphological study following chemoradiotherapy in 25 patients with Ewing's sarcoma and 14 patients with reticulum cell sarcoma. The signs of therapeutic pathomorphosis were observed in the first 3 days, and substitution of the connective tissue for a necrotized tumor started by the 3rd-4th week after the initiation of therapy. In some cases tumor growth and recurrences at the site of a treated tumor were observed.

Combined Modality Therapy↗

[Ewing sarcoma. Diagnostic and therapeutic problems in children].

A pathological X-ray of the skeleton with the clinical correlation "painful swelling of soft tissue" is always an alarming sign in childhood. Radiology is our most important device to decide if the disease is of traumatic, inflammatory, incretory or neoplasmatic as well as of degenerative origin. Considering the group of "neoplasmatic alterations" there are more than 50 different entities. The differential diagnosis of a "bone tumor" requires large experience from the radiologist. Supporting the oncologist an experienced pathologist must be capable to distinguish a reticulosarcoma, a neuroblastoma metastasis, a leukemic bone-infiltrate or an embryonal rhabdo-myosarcoma from an Ewing's sarcoma. An experienced surgeon is of the same importance capable of not only collecting detritus out of necrotic tumor centres or altered tissues, but of gathering several samples from vital tumor tissue. This experienced team is especially required when children are in concern, as this age represents the peak of manifestation of malignant bone-sarcoma as Ewing's sarcoma or osteosarcoma. The quoted case-descriptions point our how difficult it may be to find the proper biopsy location and how initial false diagnosis lets valuable time pass by and prevents early diagnosis.

Adolescent↗

[Primary reticulosarcoma of the central nervous system (author's transl)].

Among the last 7000 consecutive autopsies from our department, nine cases of primary reticulum cell sarcoma (non hodgkinian malignant lymphoma) of the central nervous system were discovered. Another case was found by cerebral biopsy. There were six males and four females (mean age 43.5 years). Mean duration of illness to time of death was 4.6 months. Gross examination revealed a wide range of neuropathological lesions (multifocal or diffusely infiltrating the parenchyma) and predominant in the cerebral hemispheres. Review of this materal underlines the difficulty of clinical diagnosis and the need to search for a more specific tratement.

Adolescent↗