Medijastinalni kaposiformni hemangioendoteliom: kratak pregled literature uz prikaz jednog novog pacijenta
Ključne reči:
kaposiformni hemangioendoteliom, Kasabah-Meritov fenomen, medijastinum, odojče
Sažetak
Kaposiformni hemangioendoteliom (KHE) je redak, lokalno invazivan, vaskularni tumor, koji je često povezan sa Kasabah-Meritovim fenomenom. Mi prikazujemo žensko, petomesečno odojče koje je na kliniku primljeno zbog dispneje, stridora i kožnih hematoma. Kompjuterizovanom tomografijom grudnog koša otkrivena je tumorska masa u medijastinumu koja se širila u veći deo levog hemitorksa, dok su laboratorijskim analizama otkriveni trombocitopenija i potrošna koagulopatija. Biopsijski uzorka tumora imao je karakterističnu mikroskopsku građu KHE sa komponentom "tufted angioma". Primenjena kortikosteroidna terapija dovela je do smanjenja volumena tumora, ali je 4 sedmice kasnije došlo do njegovog progresivnog rasta sa smrtnim ishodom uprkos dodatno primenjenoj hemioterapije. Nakon pretrage literature, pronašli smo prethodne prikaze medijastinalnog KHE kod samo 18 pacijenata, sa smrtnim ishodom bolesti kod njih 21%. Kod našeg pacijenta postojalo je nekoliko prognostički nepovoljnih elemenata bolesti: početak bolesti u ranom periodu odojčeta, velika zapremina tumora, lokacija u medijastinumu, razvoj Kasabah-Meritovog fenomena i delimičan odgovor na primenjenu terapiju.
Reference
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2. Đuričić S, Simić R. Perinatalni (fetalni i neonatalni) tumori. Materia Medica 2007, 23:26-31.
3. Aflatoon K, Aboulafia AJ, McCarthy EFJr, Frassica FJ, Levine AM. Pediatric soft-tissue tumors. J Am Acad Orthop Surg 2003; 11:332-43.
4. Charles AK. Congenital tumours. In: Keeling JW, Khong TY, editors. Fetal and Neonatal Pathology. 4th ed. London: Springer-Verlag; 2007. p. 327-78.
5. Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, et al. Vascular anomalies classification: Recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics 2015; 136:e203-14.
6. Zukerberg LR, Nickoloff BJ, Weiss SW: Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol 1993; 17:321-8.
7. Calonje E, Damaskou V, Layar AJ. Connective tissue tumors (Chapter 35). In: Calonje E, Brenn T, Lazar AJ, Billings SD, editors. McKee’s pathology of the skin with clinical correlations. 5th edition. Elsevier; 2020. p. 1698-894.
8. Goh SGN, Calonje E. Cutaneous vascular tumours: an update. Histopathology 2008; 52:661-73.
9. Lyons LL, North PE, Mac-Moune Lai F, Stoler MH, Folpe AL, Weiss SW. Kaposiform hemangioendothelioma: A study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol 2004; 28:559-68.
10. Samardzija G, Djuricic SM, Baljosevic I, Calonje E. Nasopharyngeal capillary arteriovenous malformation with ancient/symplastic change: A simulator of malignancy. Pediatr Dev Pathol 2016; 19:249-53.
11. North PE. Pediatric vascular tumors and malformations. Surg Pathol 2010;3:455-94.
12. Brasanac D, Janic D, Boricic I, Jovanovic N, Dokmanovic L. Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome. Pathol Int. 2003; 53:627-31.
13. Enjolras O, Wassef M, Mazoyer E, Frieden IJ, Rieu PN, Drouet L, et la. Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr 1997; 130:631-40.
14. Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Haberle B. Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. World J Pediatr 2018; 14:322-9.
15. Marusic Z, Billings SD. Histopathology of spindle cell vascular tumors. Surg Pathol 2017; 10:345-66.
16. Ji Y, Chen S, Peng S, Xia C, Li L. Kaposiform lymphangiomatosis and kaposiform hemagioendothelioma: similarities and differences. Orphanet J Rare Dis 2019; 14:165.
17. Ji Y, Chen S, Li L, Yang K, Li L, Yang G, et al. Kaposiform hemagioendothelioma without cutaneous involvement. J Cancer Res Clin Oncol 2018; 144:2475-84.
18. Ji Y, Yang K, Peng S, Chen S, Xiang B, Xu Z, et al. Kaposiform hemagioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon. Br J Dermatol 2018; 179:457-63.
19. Wang Z, Yao W, Sun H, Dong K, Ma Y, Chen L, et al. Sirolimus therapy for kaposiform hemangioendothelioma with long-term follow-up. J Dermatol 2019; 46:956-61.
20. Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Milliken JB, et al. Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 2013; 162:142-7.
21. Gruman A, Liang MG, Mulliken JB, Fishman SJ, Burrows PE, Kozakewich HP, et al. Kaposiform hemangioendothelioma without Kasabach-Merrit phenomenon. J Am Acad Dermatol 2005; 52:616-22.
22. Zhang G, Chen H, Gao Y, Liu Y, Wang J, Liu XY. Sirolimus for treatment of Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon: a retrospective cohort study. British J Dermatol 2018; 178:1213-4.
23. Liu T, Al-Kzayer LFY, Xie X, Fan H, Sarsam SN, Nakazawa Y, et al. Mediastinal lesions across the age spectrum: a clinicopathological comparison between pediatric and adult patients. Oncotarget 2017; 8:59845-53.
24. Wilken JJ, Meier FA, Kornstein MJ: Kaposiform hemangioendothelioma of the thymus. Arch Pathol Lab Med. 2000; 124:1542-4.
25. Wallenstein MB, Hole MK, McCarthy C, Fijalkowski N, Jeng M, Wong WB. Mediastinal Kaposiform hemangioendothelioma and Kasabach-Merritt phenomenon in a patient with no skin changes and normal chest CT. Pediatr Hematol Oncol 2014; 31:563-7.
26. Ryu YJ, Choi YH, Cheon JE, Kim WS, Kim IO, et al. Imaging findings of kaposiform hemangioendothelioma in children. Eur J Radiol 2017; 86:198-205.
27. Yasui N, Koh K, Kato M, Park M, Tomizawa D, Oshima K, et al. Kasabach-Merritt phenomenon: a report of 11 cases from a single institution. J Pediatr Hematol Oncol 2013; 35:544-8.
28. Iwami D, Shimaoka S, Mochizuki I, Sakuma T. Kaposiform hemangioendothelioma of the mediastinum in a 7-month-old boy: a case report. J Pediatr Surg. 2006; 41:1486-8.
2. Đuričić S, Simić R. Perinatalni (fetalni i neonatalni) tumori. Materia Medica 2007, 23:26-31.
3. Aflatoon K, Aboulafia AJ, McCarthy EFJr, Frassica FJ, Levine AM. Pediatric soft-tissue tumors. J Am Acad Orthop Surg 2003; 11:332-43.
4. Charles AK. Congenital tumours. In: Keeling JW, Khong TY, editors. Fetal and Neonatal Pathology. 4th ed. London: Springer-Verlag; 2007. p. 327-78.
5. Wassef M, Blei F, Adams D, Alomari A, Baselga E, Berenstein A, et al. Vascular anomalies classification: Recommendations from the International Society for the Study of Vascular Anomalies. Pediatrics 2015; 136:e203-14.
6. Zukerberg LR, Nickoloff BJ, Weiss SW: Kaposiform hemangioendothelioma of infancy and childhood. An aggressive neoplasm associated with Kasabach-Merritt syndrome and lymphangiomatosis. Am J Surg Pathol 1993; 17:321-8.
7. Calonje E, Damaskou V, Layar AJ. Connective tissue tumors (Chapter 35). In: Calonje E, Brenn T, Lazar AJ, Billings SD, editors. McKee’s pathology of the skin with clinical correlations. 5th edition. Elsevier; 2020. p. 1698-894.
8. Goh SGN, Calonje E. Cutaneous vascular tumours: an update. Histopathology 2008; 52:661-73.
9. Lyons LL, North PE, Mac-Moune Lai F, Stoler MH, Folpe AL, Weiss SW. Kaposiform hemangioendothelioma: A study of 33 cases emphasizing its pathologic, immunophenotypic, and biologic uniqueness from juvenile hemangioma. Am J Surg Pathol 2004; 28:559-68.
10. Samardzija G, Djuricic SM, Baljosevic I, Calonje E. Nasopharyngeal capillary arteriovenous malformation with ancient/symplastic change: A simulator of malignancy. Pediatr Dev Pathol 2016; 19:249-53.
11. North PE. Pediatric vascular tumors and malformations. Surg Pathol 2010;3:455-94.
12. Brasanac D, Janic D, Boricic I, Jovanovic N, Dokmanovic L. Retroperitoneal kaposiform hemangioendothelioma with tufted angioma-like features in an infant with Kasabach-Merritt syndrome. Pathol Int. 2003; 53:627-31.
13. Enjolras O, Wassef M, Mazoyer E, Frieden IJ, Rieu PN, Drouet L, et la. Infants with Kasabach-Merritt syndrome do not have “true” hemangiomas. J Pediatr 1997; 130:631-40.
14. Schmid I, Klenk AK, Sparber-Sauer M, Koscielniak E, Maxwell R, Haberle B. Kaposiform hemangioendothelioma in children: a benign vascular tumor with multiple treatment options. World J Pediatr 2018; 14:322-9.
15. Marusic Z, Billings SD. Histopathology of spindle cell vascular tumors. Surg Pathol 2017; 10:345-66.
16. Ji Y, Chen S, Peng S, Xia C, Li L. Kaposiform lymphangiomatosis and kaposiform hemagioendothelioma: similarities and differences. Orphanet J Rare Dis 2019; 14:165.
17. Ji Y, Chen S, Li L, Yang K, Li L, Yang G, et al. Kaposiform hemagioendothelioma without cutaneous involvement. J Cancer Res Clin Oncol 2018; 144:2475-84.
18. Ji Y, Yang K, Peng S, Chen S, Xiang B, Xu Z, et al. Kaposiform hemagioendothelioma: clinical features, complications and risk factors for Kasabach-Merritt phenomenon. Br J Dermatol 2018; 179:457-63.
19. Wang Z, Yao W, Sun H, Dong K, Ma Y, Chen L, et al. Sirolimus therapy for kaposiform hemangioendothelioma with long-term follow-up. J Dermatol 2019; 46:956-61.
20. Croteau SE, Liang MG, Kozakewich HP, Alomari AI, Fishman SJ, Milliken JB, et al. Kaposiform hemangioendothelioma: atypical features and risks of Kasabach-Merritt phenomenon in 107 referrals. J Pediatr 2013; 162:142-7.
21. Gruman A, Liang MG, Mulliken JB, Fishman SJ, Burrows PE, Kozakewich HP, et al. Kaposiform hemangioendothelioma without Kasabach-Merrit phenomenon. J Am Acad Dermatol 2005; 52:616-22.
22. Zhang G, Chen H, Gao Y, Liu Y, Wang J, Liu XY. Sirolimus for treatment of Kaposiform haemangioendothelioma with Kasabach-Merritt phenomenon: a retrospective cohort study. British J Dermatol 2018; 178:1213-4.
23. Liu T, Al-Kzayer LFY, Xie X, Fan H, Sarsam SN, Nakazawa Y, et al. Mediastinal lesions across the age spectrum: a clinicopathological comparison between pediatric and adult patients. Oncotarget 2017; 8:59845-53.
24. Wilken JJ, Meier FA, Kornstein MJ: Kaposiform hemangioendothelioma of the thymus. Arch Pathol Lab Med. 2000; 124:1542-4.
25. Wallenstein MB, Hole MK, McCarthy C, Fijalkowski N, Jeng M, Wong WB. Mediastinal Kaposiform hemangioendothelioma and Kasabach-Merritt phenomenon in a patient with no skin changes and normal chest CT. Pediatr Hematol Oncol 2014; 31:563-7.
26. Ryu YJ, Choi YH, Cheon JE, Kim WS, Kim IO, et al. Imaging findings of kaposiform hemangioendothelioma in children. Eur J Radiol 2017; 86:198-205.
27. Yasui N, Koh K, Kato M, Park M, Tomizawa D, Oshima K, et al. Kasabach-Merritt phenomenon: a report of 11 cases from a single institution. J Pediatr Hematol Oncol 2013; 35:544-8.
28. Iwami D, Shimaoka S, Mochizuki I, Sakuma T. Kaposiform hemangioendothelioma of the mediastinum in a 7-month-old boy: a case report. J Pediatr Surg. 2006; 41:1486-8.
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