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Spontaneous Regression Of Stage Iv Neuroblastoma

Eklöf et al., 1983Neuroblastoma

Eklöf, O., Sandstedt, B., Thönell, S., & Ahström, L. (1983). spontaneous regression of stage IV neuroblastoma. Acta paediatrica Scandinavica, 72(3), 473–476. https://doi.org/10.1111/j.1651-2227.1983.tb09752.x

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Abstract

Neuroblastoma presenting with wide spread osseous metastases (stage IV) is generally considered incurable. However, rare cases of spontaneous regression and cure are on record. This report deals with such a case in which an infant received steroids as sole treatment for developing skeletal pain. Established metastases to the brain, scalp and skeleton disappeared. Today 4 1/2 years later the patient is free from symptoms and signs of tumour.

Case Details

Disease Location

Neuroblastoma - multiple osseous metastatic lesions

Personal Characteristics

10 - months-old patented baby

Clinical Characteristics

Presented with fever, abdominal mass. An 11x7x6 cm large tumour, deriving from the right adrenal was surgically removed. No metastases. Histology: differentiated neuroblastoma. Due to age seemingly radical extirpation no adjuvant treatment was applied. Post op: staph wound infection treated with abiopsy. Readmitted 4 weeks after discharge with fever and reduced motility of the right arm. Xrays of the humerus showed a suspect periosteal reaction but a scintigram was negative. Empiric treatment with antibiotics gave temporary relief. Again readmitted with right sided exophtalmus, torticollis and multiple skeletal metastases, in addition to recurrent fever. At this point the condition was regarded incurable and the patient discharged with analgesics for skeletal pain. Due to inadequate pain relief started on daily dose of 15 mg prednisolone. In spite of continuing dissemination of osseous metastases and development of secondaries in the brain and scalp, the pain diminished, finally disappearing completely. Due to increasingly cushingoid appear-ance induced gradual withdrawal of the steroid, completed approximately 4 months after initiation of therapy and 10 months after the operation. During this period urine vma and hva values continuously increased then suddenly dropping to normal. Beginning approximately at the time of steroid withdrawal gradual healing of the metastases ensued with normalisation or near normalization of the skeletal structure. The patients physical and mental development has been completely normal. 4.5 years post diagnosis of the tumour he has been in good health without any symptoms or signs of recurrence.

Remission Characteristics

Due to increasingly cushingoid appearance induced gradual withdrawal of the steroid, completed approximately 4 months after initiation of therapy and 10 months after the operation. During this period urine vma and hva values continuously increased then suddenly dropping to normal. Beginning approximately at the time of steroid withdrawal gradual healing of the metastases ensued with normalization or near normalization of the skeletal structure. The patients physical and mental development has been completely normal. 4.5 years post diagnosis of the tumor he has been in good health without any symptoms or signs of recurrence.

Treatment & Mechanisms

Proposed Remission Mechanisms

Possible immunological mechanism

Clinical Treatment

Antibiotics for wound infection. Analgesics and steroids for pain management removal of adrenal tumor