Çocukluk Çağı Kraniyofaringiyoma Olgularının Klinik ve Laboratuvar Bulguları
Year 2020,
Volume: 18 Issue: 3, 403 - 410, 18.12.2020
Elif Söbü
,
Erdal Eren
,
Betül Sevinir
,
M.özgür Taşkapılıoğlu
Ömer Tarım
Abstract
GİRİŞ ve AMAÇ: Bu çalışmada çocukluk çağı kraniyofaringioma olgularının klinik ve laboratuar bulguları ve izlem süresince karşılaşılan endokrin sorunların değerlendirilmesi amaçlanmıştır.
YÖNTEM ve GEREÇLER: Çalışmaya Ocak 2010-Aralık 2017 arasında kraniyofaringiyoma tanısı alan olgular dahil edildi. Demografik ve klinik veriler Uludağ Üniversitesi Tıp Fakültesi Çocuk Endokrinoloji poliklinik dosyalarından retrospektif olarak değerlendirildi. İstatistiksel analizler için SPSS software version 21 kullanıldı.
BULGULAR: Sekiz yıllık süreçte tanı alan 28 olgu çalışmaya dahil edildi. Olguların yaşları 60-207 ay arasında değişmekte olup median yaş 138,5 aydı. Cinsiyet dağılımlarına bakıldığında %53 erkek(n=15) ve %47 kızdı (n=13). En sık başvuru bulguları sırayla başağrısı, görme bozuklukları ve boy kısalığıydı. Tanı anında olguların %71’inde (n: 20) tümör boyutu 3 cm’den büyüktü. Preoperatif dönemde büyüme hormonu eksikliği %17(n=5) ve hipotiroidizm %14(n=4), hipokortizolemi %10 (n=3), diyabet insipit %7,1(n=2) sıklıkta saptandı. Postoperatif dönemde olguların %89,3’ünde çoklu hipofizer hormon eksikliği saptandı.
TARTIŞMA ve SONUÇ: Kraniyofaringiyomalar yavaş büyüyen tümörler olup genellikle geç tanı alır. Geç tanı ve tümör boyutunun büyük olması sıklıkla endokrin kayıpların daha ciddi olmasına yol açar. Büyümede duraksama ve ilerleyici kilo artışı hipofizer kitlelerin erken tanısı açısından uyarıcı bulgulardır.
References
- 1. Karavitaki N, Cudlip S, Adams CBT, Wass JAH. Niki Karavitaki, Simon Cudlip, Christopher B. T. Adams, and John A. H. Wass. 2006;27(4):371–97.
- 2. Greta R. Bunin Ph.D. 1 , Tanya S. Surawicz M.P.H. 1 , Philip A. Witman M.P.H., M.Phil. 1 , Susan Preston-Martin Ph.D. 1 FDPD 1 and JMBMD 1. The descriptive epidemiology of craniopharyngioma. J Neurosurg. 1998;89(4).
- 3. L. de Vries, / L. Lazar / M. Phillip. Craniopharyngioma: presentation and endocrine sequelae in 36 children. J Pediatr Endocrinol Metab. 2003;(16):703–10.
- 4. Philippe F. Backeljauw, MD • Mehul Tulsidas Dattani, MD • Pinchas Cohen M•, Ron G. Rosenfeld M. Endocrine Disorders in Children and Adolescents. In: Mark A. Sperling, editor. Pediatric Endocrinology. 4th ed. 2014. p. 345.
- 5. Nielsen EH, Poulsgaard L, Astrup J, Bjerre P, Andersen M, Andersen C, et al. Incidence of craniopharyngioma in Denmark ( n 5 189 ) and estimated world incidence of craniopharyngioma in children and adults. 2011;755–63.
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- 7. Müller HL. Childhood Craniopharyngioma. 2013;193–202.
- 8. Massimi L, Tamburrini G, Cappa M. Long-term results of the surgical treatment of craniopharyngioma : the experience at the Policlinico Gemelli ,. 2005;(M):747–57.
- 9. Humphreys RP, Drake JM, Ch B, Smith M Lou, Ph D, Blaser SI. Aggressive surgical management of craniopharyngiomas in children. 1992;(C):47–52.
- 10. Surgical treatment of craniopharyngiomas: endocrinological results. 1999;90:251–7.
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- 13. Marisa Weiss, M.D.∗,’Correspondence information about the author M.D. Marisa Weiss1, Leslie Sutton, M.D.2, Vanessa Marcial, M.D.1, Barbara Fowble, M.D.1, Roger Packer, M.D.3, 5, Robert Zimmerman, M.D.4, Luis Schut, M.D.2, Derek Bruce, M.D.2, Giulio D’angi 5. The role of radiation therapy in the management of childhood craniopharyngioma. Int J Radiat Oncol. 1989;17(6):1313–21.
- 14. Gupta DK, Ojha BK, Sarkar C, Mahapatra AK, Sharma BS MV. Recurrence in pediatric craniopharyngiomas: analysis of clinical and histological features. Child’s Nerv Syst. 2006;22(1):50–5.
- 15. Consulting PN, Population P. Surgical management of the recurrence and regrowth of craniopharyngiomas. 2005;103:224–32.
- 16. Fahlbusch, R.; Honegger, J.; Paulus, W.; Huk, W.; Buchfelder M. Surgical treatment of craniopharyngiomas: experience with 168 patients. 1999;90:237–50.
- 17. Paper O. PROGNOSTIC FACTORS FOR TUMOR RECURRENCE AFTER GAMMA KNIFE RADIOSURGERY OF PARTIALLY RESECTED AND RECURRENT CRANIOPHARYNGIOMAS. 2012;141–8.
- 18. Cho S-KK-CW-HSCGC-K. Radical excision of pediatric craniopharyngioma: recurrence pattern and prognostic factors. Child’s Nerv Syst. 2001;17(9):531–6.
- 19. Pediatrics N, There R. Surgical management of giant pediatric craniopharyngiomas. 2010;6(November):403–16.
- 20. Juraj Š, Bízik I, Andrej Š. Craniopharyngiomas in children : how radical should the surgeon be ? 2011;41–54.
- 21. Shin TJ, Moon JK, Kang IKS, Kim J. Endocrinological Outcomes of Pediatric Craniopharyngiomas with Anatomical Pituitary Stalk Preservation : Preliminary Study.
2010;205–12.
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- 23. Patients C, Daubenbüchel AMM, Müller HL. Neuroendocrine Disorders in Pediatric. 2015;(Kraniopharyngeom 2007):389–413.
Clinical and Laboratory Follow up of Pediatric Craniopharyngioma Cases
Year 2020,
Volume: 18 Issue: 3, 403 - 410, 18.12.2020
Elif Söbü
,
Erdal Eren
,
Betül Sevinir
,
M.özgür Taşkapılıoğlu
Ömer Tarım
Abstract
INTRODUCTION: The aim of this study was to evaluate the clinical and laboratory findings and follow up of endocrine status of pediatric craniopharyngioma cases.
MATERIALS and METHODS: The patients diagnosed with craniopharyngioma between January 2010-December 2017 were included in this study. Demographic and clinical findings were retrospectively examined from medical records of Uludag University Medical Faculty Pediatric Endocinology Department. Statistical analyses were performed using the SPSS software version 21.
RESULTS: We identified a total of 28 patients with craniopharyngioma during this period. The age of the patients ranged from 60 to 207 months, with a median age of138.5months.Fifty-three percent (n=15) of cases were male and forty-seven percent (n=13) of the cases were female.The most common presenting complaints were headache, visual impairment, and short stature in decreasing order. Tumor size was greater than 3 cm in 71% (n: 20) of patients at the time of diagnosis. When the endocrine status of the patients were examined in the preoperative period, it was determined that 17% (n: 5) of the cases had growth hormone deficiency, 14% (n: 4) had hypothyroidism, 10%(n: 3) had cortisol deficiency, and 7.1% (n: 2) had diabetes insipidus. In the postoperative period, panhypopituitarism developed in 89.3% (n: 25) of the cases.
CONCLUSIONS: Craniopharyngiomas are slow-growing tumors, the tumor is usually diagnosed lateand reaches large sizes. Late diagnosis and large tumor size often lead to more serious endocrine losses. Failure to thrive and progressive weight gain are stimulating findings for early diagnosis of pituitary masses.
References
- 1. Karavitaki N, Cudlip S, Adams CBT, Wass JAH. Niki Karavitaki, Simon Cudlip, Christopher B. T. Adams, and John A. H. Wass. 2006;27(4):371–97.
- 2. Greta R. Bunin Ph.D. 1 , Tanya S. Surawicz M.P.H. 1 , Philip A. Witman M.P.H., M.Phil. 1 , Susan Preston-Martin Ph.D. 1 FDPD 1 and JMBMD 1. The descriptive epidemiology of craniopharyngioma. J Neurosurg. 1998;89(4).
- 3. L. de Vries, / L. Lazar / M. Phillip. Craniopharyngioma: presentation and endocrine sequelae in 36 children. J Pediatr Endocrinol Metab. 2003;(16):703–10.
- 4. Philippe F. Backeljauw, MD • Mehul Tulsidas Dattani, MD • Pinchas Cohen M•, Ron G. Rosenfeld M. Endocrine Disorders in Children and Adolescents. In: Mark A. Sperling, editor. Pediatric Endocrinology. 4th ed. 2014. p. 345.
- 5. Nielsen EH, Poulsgaard L, Astrup J, Bjerre P, Andersen M, Andersen C, et al. Incidence of craniopharyngioma in Denmark ( n 5 189 ) and estimated world incidence of craniopharyngioma in children and adults. 2011;755–63.
- 6. Ph D, Davis F, Ph D, Bruner JM. The descriptive epidemiology of craniopharyngioma. 1997;3(6).
- 7. Müller HL. Childhood Craniopharyngioma. 2013;193–202.
- 8. Massimi L, Tamburrini G, Cappa M. Long-term results of the surgical treatment of craniopharyngioma : the experience at the Policlinico Gemelli ,. 2005;(M):747–57.
- 9. Humphreys RP, Drake JM, Ch B, Smith M Lou, Ph D, Blaser SI. Aggressive surgical management of craniopharyngiomas in children. 1992;(C):47–52.
- 10. Surgical treatment of craniopharyngiomas: endocrinological results. 1999;90:251–7.
- 11. Mu HL. Consequences of Craniopharyngioma Surgery in Children. 2011;96(July):1981–91.
- 12. Kenneth Shapiro M.D. 1 , Kenneth Till M.A., M.B. FRCS 1 and DNGFRCS 1. Craniopharyngiomas in childhood A rational approach to treatment. J Neurosurg. 1979;50(5):617–23.
- 13. Marisa Weiss, M.D.∗,’Correspondence information about the author M.D. Marisa Weiss1, Leslie Sutton, M.D.2, Vanessa Marcial, M.D.1, Barbara Fowble, M.D.1, Roger Packer, M.D.3, 5, Robert Zimmerman, M.D.4, Luis Schut, M.D.2, Derek Bruce, M.D.2, Giulio D’angi 5. The role of radiation therapy in the management of childhood craniopharyngioma. Int J Radiat Oncol. 1989;17(6):1313–21.
- 14. Gupta DK, Ojha BK, Sarkar C, Mahapatra AK, Sharma BS MV. Recurrence in pediatric craniopharyngiomas: analysis of clinical and histological features. Child’s Nerv Syst. 2006;22(1):50–5.
- 15. Consulting PN, Population P. Surgical management of the recurrence and regrowth of craniopharyngiomas. 2005;103:224–32.
- 16. Fahlbusch, R.; Honegger, J.; Paulus, W.; Huk, W.; Buchfelder M. Surgical treatment of craniopharyngiomas: experience with 168 patients. 1999;90:237–50.
- 17. Paper O. PROGNOSTIC FACTORS FOR TUMOR RECURRENCE AFTER GAMMA KNIFE RADIOSURGERY OF PARTIALLY RESECTED AND RECURRENT CRANIOPHARYNGIOMAS. 2012;141–8.
- 18. Cho S-KK-CW-HSCGC-K. Radical excision of pediatric craniopharyngioma: recurrence pattern and prognostic factors. Child’s Nerv Syst. 2001;17(9):531–6.
- 19. Pediatrics N, There R. Surgical management of giant pediatric craniopharyngiomas. 2010;6(November):403–16.
- 20. Juraj Š, Bízik I, Andrej Š. Craniopharyngiomas in children : how radical should the surgeon be ? 2011;41–54.
- 21. Shin TJ, Moon JK, Kang IKS, Kim J. Endocrinological Outcomes of Pediatric Craniopharyngiomas with Anatomical Pituitary Stalk Preservation : Preliminary Study.
2010;205–12.
- 22. Units N. Management of childhood craniopharyngioma: can the morbidity of radical surgery be predicted? 1996;85:73–81.
- 23. Patients C, Daubenbüchel AMM, Müller HL. Neuroendocrine Disorders in Pediatric. 2015;(Kraniopharyngeom 2007):389–413.