Clinical case of primary multiple hemangioendothelioma in a child with CNS lesions
M. A. Kolcheva,
E. V. Kumirova,
M. I. Livshits,
D. A. Skobeev,
V. N. Umerenkov,
B. I. Oleynikov,
A. A. Kuznetsova,
G. E. Chmutin https://doi.org/10.21682/2311-1267-2022-9-4-88-95
Abstract
Epithelioid hemangioendothelioma (EHE) is one of the rarest vascular tumor found in the brain. In the pediatric group of patients, the proportion of EHE is only 0.02% of all brain neoplasms, and therefore oncogenesis and the causes are currently not well understood. Intracranial localization of EHE is extremely rare, nevertheless, this type of neoplasm should be included in the differential diagnostic series when multiple small intracerebral masses with hypointense MR signal in T2 mode are detected on magnetic resonance imaging. No specific therapy is currently developed for this disease, the optimal treatment is surgical removal of the volumetric mass if this type of treatment is possible. This article presents a clinical case of a primary multiple CNS lesion in a 14-year-old child with a long history of epilepsy. According to our data, the presented clinical case is the only one registered in the world, with primary multiple CNS EHE of the brain in the pediatric population
Keywords
Supporting agencies: The study was performed without external funding
About the Authors
M. A. Kolcheva
Morozovskaya Children’s Clinical Hospital of the Moscow City Healthcare Department; Peoples’ Friendship University of Russia
Russian Federation
Russian Federation
Resident in Neurosurgery
119049
1/9 4th Dobryninskiy Per.
Department of Nervous Diseases and Neurosurgery
117198
6 Miklukho-Maklaya St.
Moscow
E. V. Kumirova
Morozovskaya Children’s Clinical Hospital of the Moscow City Healthcare Department; Russian Scientific Center of Roentgenoradiology, Ministry of Health of Russia; N. N. Blokhin National Medical Research Centre of Oncology, Ministry of Health of Russia
Russian Federation
Russian Federation
Dr. of Sci. (Med.), Pediatric Oncologist, Deputy Chief Physician for Oncology, Chief Lead Officer, Scientific Consultant
119049
1/9 4<sup>th</sup> Dobryninskiy Per.
117977
86 Profsoyuznaya St.
Research Institute of Pediatric Oncology and Hematology
115478
23 Kashirskoe Shosse
Moscow
M. I. Livshits
Morozovskaya Children’s Clinical Hospital of the Moscow City Healthcare Department
Russian Federation
Russian Federation
Cand. of Sci. (Med.), Neurosurgeon, Head of the Department
Neurosurgery Department
119049
1/9 4<sup>th</sup> Dobryninskiy Per.
Moscow
D. A. Skobeev
Morozovskaya Children’s Clinical Hospital of the Moscow City Healthcare Department
Russian Federation
Russian Federation
Pathologist
Pathology Department
119049
1/9 4<sup>th</sup> Dobryninskiy Per.
Moscow
V. N. Umerenkov
Morozovskaya Children’s Clinical Hospital of the Moscow City Healthcare Department
Russian Federation
Russian Federation
Neurosurgeon
Neurosurgery Department
119049
1/9 4<sup>th</sup> Dobryninskiy Per.
Moscow
B. I. Oleynikov
Morozovskaya Children’s Clinical Hospital of the Moscow City Healthcare Department; Peoples’ Friendship University of Russia
Russian Federation
Russian Federation
Neurosurgeon, Clinical Postgraduate Student
Neurosurgery Department
119049
1/9 4<sup>th</sup> Dobryninskiy Per.
Department of Nervous Diseases and Neurosurgery
117198
6 Miklukho-Maklaya St.
Moscow
A. A. Kuznetsova
Morozovskaya Children’s Clinical Hospital of the Moscow City Healthcare Department; Scientific and Practical
Psychoneurological Center named after Z. P. Solovyov of the Moscow City Healthcare Department
Russian Federation
Russian Federation
Neurologist, Junior Researcher
Neurosurgery Department
119049
1/9 4<sup>th</sup> Dobryninskiy Per.
115419
43 Donskaya St.
Moscow
G. E. Chmutin
Morozovskaya Children’s Clinical Hospital of the Moscow City Healthcare Department; Peoples’ Friendship University of Russia
Russian Federation
Russian Federation
Dr. of Sci. (Med.), Professor, Neurosurgeon, Head of Service, Head of Department
Neurosurgical Service
119049
1/9 4<sup>th</sup> Dobryninskiy Per.
6 Miklukho-Maklaya St.
Department of Nervous Diseases and Neurosurgery
117198
Moscow
References
1. Weiss S. W., Enzinger F. M. Epitheloid hemangioendothelioma. A vascular tumor often mistaken for a carcinoma. Cancer. 1982; 50 (5): 970–81. doi: 10.1002/1097-0142(19820901)50:5<970::aid-cncr2820500527>3.0.co;2-z.
2. Louis D. N., Ohgaki H., Wiestier O. D., Cavenee W. K., Ellison D. W., Figarella-Branger D., editors. WHO Classifi cation of Tumours of the Central Nervous System (IARC WHO Classification of Tumours), revised 4<sup>th</sup> edn., Agency for Research on Cancer, World Health Organization 2016-05-13, Pp. 11, 258. doi: 10.1007/s00401-016-1545-1.
3. Díaz R., Segura Á., Calderero V., Cervera I., Aparicio J., Jordá M. V., Pellín L. Central nervous system metastases of a pulmonary epitheloid haemangioendothelioma. Eur Respiratory J. 2004; 23: 483–6. doi: 10.1183/09031936.04.00060104.
4. Endo T., Su C. C., Numagami Y., Shirane R. Malignant intracranial epithelioid hemangioendothelioma presumably originating from the lung: Case report. J Neurooncol. 2004; 67: 337–43. PMID: 23759830.
5. Fernandes A. L., Ratilal B., Mafra M., Magalhaes C. Aggressive intracranial and extra-cranial epithelioid hemangioendothelioma: A case report and review of the literature. Neuropathology. 2006; 26: 201–5. doi: 10.11477/mf.1436203671.
6. Gu H. L., Zeng S. X., Chang Y. B., Lin Z., Zheng Q. J., Zheng X. Q., Peng Z. W., Zhan S. Q. Multidisciplinary treatment based on surgery leading to long-term survival of a patient with multiple asynchronous rare primary malignant neoplasms: a case report and literature review. Oncol Lett. 2015; 9 (3): 1135–41. doi: 10.3892/ol.2014.2833.
7. Soape M. P., Verma R., Payne J. D., Wachtel M., Hardwicke F., Cobos E. Treatment of hepatic epithelioid hemangioendothelioma: finding uses for thalidomide in a new era of medicine. Case Rep Gastrointest Med. 2015; 2015: 326795. doi: 10.1155/2015/326795.
8. Engel E. R., Cournoyer E., Adams D. M., Stapleton S. A retrospective review of the use of sirolimus for pediatric patients with epithelioid hemangioendothelioma. J Pediatr Hematol Oncol. 2020; 42: e826–9. PMID: 31714437. doi: 10.1097/MPH.0000000000001643.
9. Rosenberg A., Agulnik M. Epithelioid Hemangioendothelioma: Update on Diagnosis and Treatment. Curr Treat Options Oncol. 2018; 19 (4): 19. doi: 10.1007/s11864-018-0536-y.
10. Ji Y., Chen S., Yang K., Xia C., Li L. Kaposiform hemangioendothelioma: current knowledge and future perspectives. Orphanet J Rare Dis. 2020; 15 (1): 39. doi: 10.1186/s13023-020-1320-1.
11. Fryer J. A., Biggs M. T., Katz I. A., Brazier D. H., Shakespeare T. P. Intracranial epithelioid hemangioendothelioma arising at site of previously excised atypical meningioma. Pathology. 1998; 30 (2): 95–9. doi: 10.1080/00313029800169026.
12. Baehring J. M., Dickey P. S., Bannykh S. I. Epithelioid hemangioendothelioma of the suprasellar area: A case report and review of the literature. Arch Pathol Lab Med. 2004; 128: 1289–93. PMID: 27656318.
13. Chan Y. L., Ng H. K., Poon W. S., Cheung H. S. Epithelioid haemangioendothelioma of the brain: A case report. Neuroradiology. 2001; 43: 848–50. PMID: 11688702.
14. Chen T. C., Gonzalez-Gomez I., Gilles F. H., McComb J. G. Pediatric intracranial hemangioendotheliomas: Case report. Neurosurgery. 1997; 40: 410–4. PMID: 9007881.
15. Chow L. T., Chow W. H., Fong D. T. Epithelioid hemangioendothelioma of the brain. Am J Surg Pathol. 1992; 16: 619–25. PMID: 1376023.
16. Hodaie M., Becker L., Teshima I., Rutka J. T. Total resection of an intracerebral hemangioendothelioma in an infant. Case report and review of the literature. Pediatr Neurosurg. 2001; 34: 104–12. PMID: 11287811. doi: 10.1159/000056003.
17. Llena J. F., Hirano A., Inoue A. Vasoformative tumor of the brain – Immunohistology and ultrastructure. Clin Neuropathol. 1984; 3: 155–9. PMID: 6434214.
18. Mohan S. M., Symss N. P., Pande A., Chakravarthy V. M., Ramamurthi R. Intracranial epithelioid hemangioendothelioma. Childs Nerv Syst. 2008; 24: 863–8. PMID: 15218955. doi: 10.1023/b:neon.0000027760.84461.c7.
19. D’Annibale M., Piovanello P., Carlini P., Del Nonno F., Sciarretta F., Rossi M., Berloco P., Iappelli M., Lonardo M. T., Perrone R., Donnorso R. Epithelioid hemangioendothelioma of the liver: case report and review of the literature. Transplant Proc. 2002; 34: 1248–51. doi: 10.1016/s0041-1345(02)02751-3.
20. Gelin M., Van de Stadt J., Rickaert F., Adler M., Lambilliotte J. P. Epithelioid hemangioendothelioma of the liver following contact with vinyl chloride. Recurrence after orthotopic liver transplantation. J Hepatol. 1989; 8 (1): 99–106. doi: 10.1016/0168-8278(89)90168-2.
21. Golash A., Strang F. A., Reid H. Intracranial haemangioendothelioma mimicking a meningioma. Br J Neurosurg. 1999; 13: 594–7. PMID: 10715730. doi: 10.1080/02688699943123.
22. Hamlat A., Casallo-Quilliano C., Saikali S., Lesimple T., Brassier G. Epithelioid hemangioendothelioma of the infundibular-hypothalamic region: Case report and literature review. J Neurooncol. 2004; 67: 361–6. PMID: 15164993. doi: 10.1023/b:neon.0000024240.97378.d0.
23. Hurley T. R., Whisler W. W., Clasen R. A., Smith M. C., Bleck T. P., Doolas A., Dampier M. F. Recurrent intracranial epithelioid hemangioendothelioma associated with multicentric disease of liver and heart: Case report. Neurosurgery. 1994; 35: 148–51. PMID: 7936138. doi: 10.1227/00006123-199407000-00024.
24. Kitaichi M., Nagai S., Nishimura K., Itoh H., Asamoto H., Izumi T., Dail D. H. Pulmonary epithelioid haemangioendothelioma in 21 patients, including three with partial spontaneous regression. Eur Respir J. 1998; 12: 89–96. PMID: 9701420. doi: 10.1183/09031936.98.12010089.
25. Kubota T., Sato K., Takeuchi H., Handa Y. Successful removal after radiotherapy and vascular embolization in a huge tentorial epithelioid hemangioendothelioma: A case report. J Neurooncol. 2004; 68: 177–83. PMID: 15218955. doi: 10.1023/b:neon.0000027760.84461.c7.
26. Louis D. N., Ohgaki H., Wiestler O. D., Cavenee W. K., Burger P. C., Jouvet A., Scheithauer B. W., Kleihues P. The 2007 WHO Classification of Tumours of the Central Nervous System. Acta Neuropathol. 2007; 114: 97–109. doi: 10.1007/s00401-007-0243-4.
27. Mistry A. M., Gorden D. L., Busler J. F., Coogan A. C., Kelly B. S. Diagnostic and therapeutic challenges in hepatic epithelioid hemangioendothelioma. J Gastrointest Cancer. 2012; 43: 521–5. PMID: 22544493. doi: 10.1007/s12029-012-9389-y.
28. Zheng J., Liu L., Wang J., Wang S., Cao Y., Zhao J. Primary intracranial epithelioid hemangioendothelioma: A low-proliferation tumor exhibiting clinically malignant behavior. J Neurooncol. 2012; 110: 119–27. doi: 10.1007/s11060-012-0945-x.
29. Tammam A. G., Lewis P. D., Crockard H. A. Cerebellopontine angle epithelioid haemangioendothelioma in a 4-year-old boy. Childs Nerv Syst. 1997; 13: 648–50. PMID: 9454988. doi: 10.1007/s003810050162.
30. Taratuto A. L., Zurbriggen G., Sevlever G., Saccoliti M. Epithelioid hemangioendothelioma of the central nervous system. Immunohistochemical and ultrastructural observations of a pediatric case. Pediatr Neurosci. 1998; 14: 11–4. PMID: 3217280. doi: 10.1159/000120355.
31. Venizelos I. D., Paradinas F. J. Primary paediatric intracranial epithelioid haemangioendothelioma. Histopathology. 2002; 41: 172–4. PMID: 12147098. doi: 10.1046/j.1365-2559.2002.01424_3.x.
32. Aniba K., Laghmari M., Lmejjati M., Ghannane H., Ait Benali S. A tragical paediatric case history of intraorbital and intracranial epithelioid hemangioendothelioma. Case Rep Neurol Med. 2012; 2012: 396097. doi: 10.1155/2012/396097. PMID: 23133764.
33. Frota Lima L. M., Packard A. T., Broski S. M. Epithelioid hemangioendothelioma: evaluation by 18 F-FDG PET / CT. Am J Nucl Med Mol Imaging. 2021; 11 (2): 77–86. PMID: 34079637.
34. Parajón A., Vaquero J. Meningel intracranial epithelioid hemangioendothelioma: Case report and literature review. J Neurooncol. 2008; 88: 169–73. PMID: 18278439. doi: 10.1007/s11060-008-9543-3.
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For citations:
Kolcheva M.A., Kumirova E.V., Livshits M.I., Skobeev D.A., Umerenkov V.N., Oleynikov B.I., Kuznetsova A.A., Chmutin G.E. Clinical case of primary multiple hemangioendothelioma in a child with CNS lesions. Russian Journal of Pediatric Hematology and Oncology. 2022;9(4):88-95. (In Russ.) https://doi.org/10.21682/2311-1267-2022-9-4-88-95
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