Hemangioendotelioma maligno do crânio: relato de um caso em criança

Pereira, Walter C.; Lopes, Pedro G.; Chagas, Antonio J.; Lefèvre, Antonio B.; Tenuto, Rolando A.; Mattosinho-França, L. C.

doi:10.1590/S0004-282X1969000400012

Resumos

Os hemangioendoteliomas primitivamente ósseos são neoplasias muito raras e de diagnósticos clínico e radiológico difíceis. Em virtude de seu crescimento rápido, grande capacidade infiltrativa e metastatização precoce, o prognóstico é geralmente sombrio, mesmo com cirurgia radical seguida de radioterapia. Relatamos um caso de hemangioendotelioma maligno, localizado no crânio, em criança de 6 anos de idade, cujo diagnóstico radiológico foi de displasia fibrosa óssea. O doente foi operado, sendo feita exérese aparentemente total da neoplasia e, a seguir, irradiado com cobaltoterapia em doses elevadas; tais medidas, contudo, não impediram a progressão do processo neoplásico. A quimioterapia, com Enduxan intravenoso, determinou a cura clínica e radiológica do paciente. São discutidos varios aspectos referentes ao diagnóstico do hemangioendotelioma maligno, tendo em vista a grande confusão existente na literatura em relação à nomenclatura e classificação desses tumores.

The primary localization of hemangioendothelioma in the bone is very rare and the clinical and radiological diagnosis is often difficult. Due to the rapid growth, invasive properties and early metastatization, the evolution of these neoplasms is often progressive, despite the radical removal followed by radiotherapy. A case of a malignant hemangioendothelioma of the skull in a 6-year-old male patient is reported. The radiological aspects supported the diagnosis of fibrous dysplasia of bone. The patient was operated on, and an apparently total removal of the tumor was performed, followed by cobaltotherapy; however, the growth of the neoplasm was not arrested. Thus, chemotherapy with intravenous Enduxan (50 mg per day) was employed during a month, with clinical recovery and radiological cure of the process. Some clinical features on the differential diagnosis of hemangioendothelioma are discussed due to the confusion on the terminology and classification of these tumors.

Hemangioendotelioma maligno do crânio: relato de um caso em criança

Malignant hemangioendothelioma of the skull in a child

Walter C. Pereira^I; Pedro G. Lopes^II; Antonio J. Chagas^II; Antonio B. Lefèvre^III; Rolando A. Tenuto^IV; L. C. Mattosinho-França^IV

^IDepartamento de Neurologia da Faculdade de Medicina da Universidade de São Paulo (Prof. Adherbal Tolosa): Neurocirurgião

^IIDepartamento de Neurologia da Faculdade de Medicina da Universidade de São Paulo (Prof. Adherbal Tolosa): Estagiário

^IIIDepartamento de Neurologia da Faculdade de Medicina da Universidade de São Paulo (Prof. Adherbal Tolosa): Professor de Disciplina de Neuropediatria; ^IV Professor de Disciplina de Neurocirurgia

^IVDepartamento de Neurologia da Faculdade de Medicina da Universidade de São Paulo (Prof. Adherbal Tolosa): Patologista

RESUMO

Os hemangioendoteliomas primitivamente ósseos são neoplasias muito raras e de diagnósticos clínico e radiológico difíceis. Em virtude de seu crescimento rápido, grande capacidade infiltrativa e metastatização precoce, o prognóstico é geralmente sombrio, mesmo com cirurgia radical seguida de radioterapia.

Relatamos um caso de hemangioendotelioma maligno, localizado no crânio, em criança de 6 anos de idade, cujo diagnóstico radiológico foi de displasia fibrosa óssea. O doente foi operado, sendo feita exérese aparentemente total da neoplasia e, a seguir, irradiado com cobaltoterapia em doses elevadas; tais medidas, contudo, não impediram a progressão do processo neoplásico. A quimioterapia, com Enduxan intravenoso, determinou a cura clínica e radiológica do paciente. São discutidos varios aspectos referentes ao diagnóstico do hemangioendotelioma maligno, tendo em vista a grande confusão existente na literatura em relação à nomenclatura e classificação desses tumores.

SUMMARY

The primary localization of hemangioendothelioma in the bone is very rare and the clinical and radiological diagnosis is often difficult. Due to the rapid growth, invasive properties and early metastatization, the evolution of these neoplasms is often progressive, despite the radical removal followed by radiotherapy.

A case of a malignant hemangioendothelioma of the skull in a 6-year-old male patient is reported. The radiological aspects supported the diagnosis of fibrous dysplasia of bone. The patient was operated on, and an apparently total removal of the tumor was performed, followed by cobaltotherapy; however, the growth of the neoplasm was not arrested. Thus, chemotherapy with intravenous Enduxan (50 mg per day) was employed during a month, with clinical recovery and radiological cure of the process. Some clinical features on the differential diagnosis of hemangioendothelioma are discussed due to the confusion on the terminology and classification of these tumors.

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Departamento de Neurologia Faculdade de Medicina da Universidade de São Paulo Caixa Postal 3461 São Paulo, SP Brasil.

1. BOENHEIM, F. & Mc GAVACH, T. H. Polyostotische fibrose dysplasie. Ergebn. inn. Med. Kinderheilk. 3:157, 1952.
2. BOTSFORD, T. W. Tumors of the small intestine: a review of experience with 115 cases including a report of a rare case of malignant hemangioendothelioma. Amer. J. Surg. 103:358, 1962.
3. CABANNE, F.; MICHIELS, R.; DUSSERRE, P. & TUAILLON, P. Hémangioendothéliomes du sein: étude anatomo-clinique de deux cas. Laval Med. 37:579, 1966.
4. CARTER, J. H.; DICKERSON, R. & NEEDY, C. Angiosarcoma of bone: a review of the literature and presentation of a case. Ann. Surg. 144:107, 1956.
5. CATEL, W. Diagnostico en Pediatria. Salvat, Barcelona, 2.Ş ed., 1966.
6. FREILICH, L. B. & COE, G. C. Angiosarcoma: case reporte and review of the literature. Amer. J. Cancer, 26:269, 1936.
7. GLASSER, A. & REICHMANN, J. Multizentrisches unilaterales hämangioendothelioma des hand-arm-skeletts. Brun's Beitr. klin Chir. 210:422, 1965.
8. HAUSER, E. D. W. & CONSTANT, G. A. Skeletal hemangioendothelioma: a case report. J. Bone Jt Surg. 30A :517, 1948.
9. HENRY, F. A. Angiosarcoma of maxila in three month old infant. J. Oral Surg. 7:250, 1949.
10. HUECK, W. Morphologische Pathologie. Georg Thieme Verlag, Leipzig, 1937.
11. JAFFE, H. L. Fibrous dysplasia of bone. Bull. N. Y. Acad. Med. 22:588, 1948.
12. JAFFE, H. L. Tumors and Tumorous Conditions of the Bones and Joints. Lea & Febiger, Philadelphia, ed. 2, 1961.
13. LICHTENSTEIN, L. Bone Tumors. Mosby, St. Louis, ed. 2, 1959.
14. LICHTENSTEIN, L. & JAFFE, H. L. Fibrous dysplasia of bone: a condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extra-skeletal abnormalities. Arch. Path. 33:777, 1942.
15. Mc CARTHY, W. D. & PACK, G. T. Malignant blood vessel tumors: 56 cases of angiosarcoma and Kaposi's sarcoma. Surg. Gynec. Obstet. 91:456, 1950.
16. PINHEIRO, L. C. Da quimioterapia antineoplásica em circuito isolado. Rev. brasil. Cirurg. 45:252, 1963.
17. POLLAK, A. Angiosarcoma of the sternun. Amer. J. Surg. 77:522, 1949.
18. RAMSEY, H. J. Fine structure of the hemangiopericytoma and hemangioendothelioma. Cancer 19:2005, 1966.
19. ROBBINS, S. L. Textbook of Pathology, W. B. Saunders, Philadelphia, ed. 2, 1962.
20. STOUT, A. P. Hemangioendothelioma: a tumor of blood vessels featuring endothelial cells. Ann. Surg. 118:445, 1943.
21. STOUT, A. P. Tumors of the soft tissue. Armed Forces Institute of Pathology, Washington, 1953.
22. THOMAS, A. Vascular tumors of bone: a pathological and clinical study of 27 cases. Surg. Gynec. Obstet. 75:777, 1942.
23. TURNER, O. A. & KERNOHAN, J. W. Vascular malformations and vascular tumors involving the spinal cord: a pathological study of 46 cases. Arch. Neurol. Psychiat. 46:444, 1941.
24. VIGO, N. J.; SCHAPOSNIK, F. & LAGUENS, R. Renal hemangioendothelioma. Rev. argent. Urol. 33:107, 1964.

Datas de Publicação

Publicação nesta coleção
23 Abr 2013
Data do Fascículo
Dez 1969

This work is licensed under a Creative Commons Attribution-NonCommercial 4.0 International License.

[1] 1. BOENHEIM, F. & Mc GAVACH, T. H. Polyostotische fibrose dysplasie. Ergebn. inn. Med. Kinderheilk. 3:157, 1952.

[2] 2. BOTSFORD, T. W. Tumors of the small intestine: a review of experience with 115 cases including a report of a rare case of malignant hemangioendothelioma. Amer. J. Surg. 103:358, 1962.

[3] 3. CABANNE, F.; MICHIELS, R.; DUSSERRE, P. & TUAILLON, P. Hémangioendothéliomes du sein: étude anatomo-clinique de deux cas. Laval Med. 37:579, 1966.

[4] 4. CARTER, J. H.; DICKERSON, R. & NEEDY, C. Angiosarcoma of bone: a review of the literature and presentation of a case. Ann. Surg. 144:107, 1956.

[5] 5. CATEL, W. Diagnostico en Pediatria. Salvat, Barcelona, 2.Ş ed., 1966.

[6] 6. FREILICH, L. B. & COE, G. C. Angiosarcoma: case reporte and review of the literature. Amer. J. Cancer, 26:269, 1936.

[7] 7. GLASSER, A. & REICHMANN, J. Multizentrisches unilaterales hämangioendothelioma des hand-arm-skeletts. Brun's Beitr. klin Chir. 210:422, 1965.

[8] 8. HAUSER, E. D. W. & CONSTANT, G. A. Skeletal hemangioendothelioma: a case report. J. Bone Jt Surg. 30A :517, 1948.

[9] 9. HENRY, F. A. Angiosarcoma of maxila in three month old infant. J. Oral Surg. 7:250, 1949.

[10] 10. HUECK, W. Morphologische Pathologie. Georg Thieme Verlag, Leipzig, 1937.

[11] 11. JAFFE, H. L. Fibrous dysplasia of bone. Bull. N. Y. Acad. Med. 22:588, 1948.

[12] 12. JAFFE, H. L. Tumors and Tumorous Conditions of the Bones and Joints. Lea & Febiger, Philadelphia, ed. 2, 1961.

[13] 13. LICHTENSTEIN, L. Bone Tumors. Mosby, St. Louis, ed. 2, 1959.

[14] 14. LICHTENSTEIN, L. & JAFFE, H. L. Fibrous dysplasia of bone: a condition affecting one, several or many bones, the graver cases of which may present abnormal pigmentation of skin, premature sexual development, hyperthyroidism or still other extra-skeletal abnormalities. Arch. Path. 33:777, 1942.

[15] 15. Mc CARTHY, W. D. & PACK, G. T. Malignant blood vessel tumors: 56 cases of angiosarcoma and Kaposi's sarcoma. Surg. Gynec. Obstet. 91:456, 1950.

[16] 16. PINHEIRO, L. C. Da quimioterapia antineoplásica em circuito isolado. Rev. brasil. Cirurg. 45:252, 1963.

[17] 17. POLLAK, A. Angiosarcoma of the sternun. Amer. J. Surg. 77:522, 1949.

[18] 18. RAMSEY, H. J. Fine structure of the hemangiopericytoma and hemangioendothelioma. Cancer 19:2005, 1966.

[19] 19. ROBBINS, S. L. Textbook of Pathology, W. B. Saunders, Philadelphia, ed. 2, 1962.

[20] 20. STOUT, A. P. Hemangioendothelioma: a tumor of blood vessels featuring endothelial cells. Ann. Surg. 118:445, 1943.

[21] 21. STOUT, A. P. Tumors of the soft tissue. Armed Forces Institute of Pathology, Washington, 1953.

[22] 22. THOMAS, A. Vascular tumors of bone: a pathological and clinical study of 27 cases. Surg. Gynec. Obstet. 75:777, 1942.

[23] 23. TURNER, O. A. & KERNOHAN, J. W. Vascular malformations and vascular tumors involving the spinal cord: a pathological study of 46 cases. Arch. Neurol. Psychiat. 46:444, 1941.

[24] 24. VIGO, N. J.; SCHAPOSNIK, F. & LAGUENS, R. Renal hemangioendothelioma. Rev. argent. Urol. 33:107, 1964.