ABSTRACT
The authors report the case of a 4-year-old boy with a diagnosis of stage IV neuroblastoma (NB), who had been treated with 6 cycles of cyclophosphamide, doxorubicin, cisplatin, and etoposide for 12 months. The patient reached partial remission and presented a diagnosis of acute myelomonocytic leukemia (M4 AML), confirmed by immunophenotyping. After 2 months of therapy for leukemia, the child died with both malignancies in activity. A necropsy histologically confirmed the simultaneity of the two diseases. The authors review the possibilities of this association. The review leads to the conclusion that AML can occur as a secondary malignancy after the onset of the neuroblastoma, or be suggested by a misdiagnosis. The simultaneous occurrence of both as described here is not, however, found in the literature, to the best of the authors' knowledge.
Subject(s)
Leukemia, Myelomonocytic, Acute/etiology , Neoplasms, Second Primary/diagnosis , Neuroblastoma/drug therapy , Antineoplastic Agents, Alkylating/administration & dosage , Antineoplastic Agents, Alkylating/toxicity , Antineoplastic Combined Chemotherapy Protocols/administration & dosage , Antineoplastic Combined Chemotherapy Protocols/toxicity , Bone Marrow/pathology , Child, Preschool , Fatal Outcome , Humans , Immunohistochemistry , Immunophenotyping , Leukemia, Myelomonocytic, Acute/diagnosis , Leukemia, Myelomonocytic, Acute/pathology , Male , Neoplasms, Second Primary/etiology , Neoplasms, Second Primary/pathology , Neuroblastoma/diagnosis , Neuroblastoma/pathology , Neutropenia/etiologyABSTRACT
Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less than 1 to 5 percent of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7 percent), protein-losing enteropathy (33.3 percent) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management
Subject(s)
Female , Humans , Child, Preschool , Digestive System/pathology , Histiocytosis, Langerhans-Cell/pathology , Protein-Losing Enteropathies/pathology , Biopsy , Fatal Outcome , Hypoaldosteronism/complications , Protein-Losing Enteropathies/diagnosisABSTRACT
Symptomatic involvement of the gastrointestinal (GI) tract as a prominent symptom in Langerhans' cell histiocytosis (LCH) is uncommon, occurring in less than 1 to 5% of all cases, even when the disease is in its disseminated form. Up to now, there have been reports of 18 cases of LCH with GI manifestations, including our 2 cases, with diarrhea (77.7%), protein-losing enteropathy (33.3%) and bloody stool being the most frequent findings. The authors present two patients with severe diarrhea and refractory hypoalbuminemia, and with the protein-losing enteropathy documented by Cr51-labeled albumin studies. A review of the literature indicated that the presence of GI symptoms is often associated with systemic disease as well as with poor prognosis, mainly under 2 years of age. Radioisotopes are useful for documenting protein loss in several diseases with high specificity and sensitivity, and their utilization in the cases reviewed here permitted diagnoses in 6 children, as well as improved therapeutic management.
