Granados SE, Rocha AJ, Lázaro VN, Zepeda CR, Trejo PA, Morelos GR, Casian CGA

Language: Spanish
References: 13
Page: 61-65
PDF size: 135.07 Kb.

ABSTRACT

Purpose: Presenting histiocytosis x (Langerhans cells) as the first cause of multi-systemic lesions that significantly affect the mastoidea region, the neck, thorax and abdomen, surpassing Rabdomiosarcoma or malignant lymphoma in pediatric patients. It has been shown that the use of Computerized Tomography (CT) is highly specific and very sensitive as diagnosis method.
Material and methods: A one-and-a-half year old pediatric patient was assessed with high-resolution, helicoidal computerized tomography with 5 mm cuts, and a non-ionic endovenous contrast. In mastoid, neck, thorax and abdomen, multifrequency ultrasound was used to show all those places where histiocytosis x was found. Byopsy through the pathology service confirmed the diagnosis.
Results: The most significant lesion leading to consultation was the one found in the ear, where the temporal bone was found destroyed and there was invasion into the nervous system towards the temporal lobe. Based on the characteristics found, a tomography was taken on neck, thorax and abdomen where multiple infiltration lesions were found in the rib archs and neck.
Conclusion: The type of bone destruction found in the mastoid made physicians think that it could very possibly be a rabdomiosarcoma, only the presence of multiple lesions and the hystopathologic study would show that it was histiocytosis x.

REFERENCES

1. Fernandez-Latorre F, Menor-Serrano FS, Alonso-Charterina J, Arenas-Jimenez. Langerhans´cell histiocytosis of the temporal bone in pediatric patients: imagin and follow-up. AJR 2000; 174: 217-21.

2. Bain ES, Kinney TB, Gooding JM, Casasola G, Ysrael MZ. Sinus histiocytosis with massive lymphadenopatia (rosai-dorfman disease): a rare causa of bilateral renal masses. AJR 1999; 172: 995-6.

3. Siegelman SS. Taking the X out of histiocitosis Y. Radiology 1997; 204: 322-4.

4. The french langerhans´cell study group. A multicentre retrospective survey of langerhans´cell histiocytosis: 348 casos observed between 1983 and 1993. Arch child 1996; 75: 17-24.

5. Beltran J, Aparisi F, Bonmati IM, Rosemberg ZS, Present D, Steiner GC. Eosinophilic granuloma: MRI manifestations. Skeletal Radiol 1993; 22: 157-61.

6. Anonsen CK, Donaldson S. Langerhans´cell histiocytosis of de head and neck. Laryngoscope 1987; 97: 537-41.

7. Mccaffrey TV, Mcdonald TJ. Histiocytosis X of de ear and temporal bone: review of 22 cases. Laryngoscope 1979; 89: 1735-42.

8. Clark RI, Margulies SI, Mulholland JH. Histiocytosis X. A fatal case with unusual pulmonary manifestations. Radiology 1970: 95: 631-2.

9. Radiology, vol 151, 35-38, copyright 1984 by Radiological Society of North America.

10. Kulwiec EL, Lynch DA, Aguayo SM, Schwarz MI, King TE Jr. Department of Radiology, University of Colorado Health Sciences Center, Denver 80262.

11. Komp DM. The treatment of sinus histiocitocys with massive lymphadenopathy (rosai dorfman disease): report of a case with widespread nodal and extranodal dissemination. Histopatology 1981; 5: 697-709.

12. Afzal M, Baez-Giangreco A, AL Jaser AN, Onoura VC. Unusual bilateral renal histiocitocys: extranodal variant of rosai-dorfman disease. Arch Pathol Lab Med 1992; 116: 1366-7.

13. Rosai J, Dorfman RF. Sinus histiocytosis uit massive lymphadenopathy: a pseudolymphomatous beningn disorder-analysis of 34 casos. Cancer 1972; 30: 1174-88.