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Xanthogranuloma of the sellar region: a systematic review

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Abstract

Knowledge of xanthogranuloma (XG) of the sellar region comes from short series or single cases. We performed a systematic review, using the PubMed, Web of Science, Embase, Scopus, eLibrary, and BIOSIS Preview databases, of all cases reported from 2000 to the present. We also describe one unreported patient treated in our institution. A search of the literature revealed that of 71 patients 50.7% were male and that mean age at diagnosis was 34.7 ± 19.2 years old. Median time from clinical onset until diagnosis was 7 (3–21) months. Hypopituitarism (70.4%), visual disorders (64.7%), headache (53.5%), and polyuria-polydipsia (28.2%) were the most common symptoms. On MRI, median tumor size was 20 (16–29) mm, while 71.8% were sellar/suprasellar and less frequently exclusively suprasellar (15.5%) or sellar (12.7%). On T1-weighted imaging, XG was hyperintense in 76.3% of patients, while it showed variable appearance on T2-weighted imaging. The tumor showed cystic features in 50.7%, gadolinium enhancement in 45.1%, and calcification in 22.5% of patients. All patients underwent surgery (77.4% transphenoidal approach and 18.3% craniotomy), with hypopituitarism (56.4%), diabetes insipidus (34.5%), and visual defects (7.3%) being the most common complications. Total/subtotal resection was achieved in 93.5%, while the tumor was partially removed in 6.6%. Median follow-up was 24 (6–55) months and no tumor recurrence or remnant growth was reported in 97.5% of cases. In conclusion, XG affects the younger population, manifested by hormonal deficit and mass effect symptoms. Surgery is safe and offers excellent outcomes, though hypopituitarism is frequent post-surgery. Tumor recurrence or remnant growth is rare and radiological surveillance is a good option for patients with remnant lesions.

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The datasets generated and/or analyzed during the current study are available from the corresponding author on reasonable request.

References

  1. Lopes MBS, Asa SL, Kleinschmidt-DeMasters, BK, Mete O, Osamura RY, Villa C (2021) Pituitary adenoma/pituitary neuroendocrine tumour. In: International Agency for Research on Cancer, 5th ed, vol 6. Central Nervous System Tumours, Lyon, pp 406–14. https://publications.iarc.fr/601

  2. Melmed S (2020) Pituitary-tumor endocrinopathies. N Engl J Med 382:937–950. https://doi.org/10.1056/NEJMra1810772

    Article  CAS  PubMed  Google Scholar 

  3. Kaltsas GA, Kolomodi D, Randeva H, Grossman A (2019) Nonneuroendocrine neoplasms of the pituitary region. J Clin Endocrinol Metab 104:3108–3123. https://doi.org/10.1210/jc.2018-01871

    Article  PubMed  Google Scholar 

  4. Famini P, Maya MM, Melmed S (2019) Pituitary magnetic resonance imaging for sellar and parasellar masses: ten-year experience in 2598 patients. J Clin Endocrinol Metab 96:1633–1641. https://doi.org/10.1210/jc.2011-0168

    Article  CAS  Google Scholar 

  5. Isaacson B (2015) Cholesterol granuloma and other petrous apex lesions. Otolaryngol Clin North Am 48:361–373. https://doi.org/10.1016/j.otc.2014.12.009

    Article  PubMed  Google Scholar 

  6. WHO classification of tumours Editorial Board (2021) International Agency for Research on Cancer, 5th ed, vol 6. Central Nervous System Tumours, Lyon. https://publications.iarc.fr/601

  7. Ahmetgjekaj I, Harizi E, Rahman A, Hyseni F, Nasir F, Decka A, Rahman M, Shemsi K, Saliaj K, Akram S, Kola I, Musa J (2022) Giant cholesterol granuloma of petrous apex. Radiol Case Rep 17:1220–1224. https://doi.org/10.1016/j.radcr.2021.12.046

    Article  PubMed  PubMed Central  Google Scholar 

  8. Shirataki K, Okada S, Matsumoto S (1988) Histopathological study of the ‘“cholesterol granuloma reaction”’ in the sellar and juxtasellar tumors. No To Shinkei 40:133–139

    CAS  PubMed  Google Scholar 

  9. Nagatani K, Waga S, Takeuchi J, Takebe Y, Hohda K, Hirano A (1990) Multiple intracranial xanthogranulomas-case report. Neurol Med Chir (Tokyo) 30:960–965. https://doi.org/10.2176/nmc.30.960

    Article  CAS  PubMed  Google Scholar 

  10. Paulus W, Honegger J, Keyvani K, Fahlbusch R (1999) Xanthogranuloma of the sellar region: a clinicopathological entity different from adamantinomatous craniopharyngioma. Acta Neuropathol 97:377–382. https://doi.org/10.1007/s004010051001

    Article  CAS  PubMed  Google Scholar 

  11. Kleihues P, Louis DN, Scheithauer BW, Rorke LB, Reifenberger G, Burger PC, Cavenee WK (2002) The WHO classification of tumors of the nervous system. J Neuropathol Exp Neurol 61:215–225; discussion 226–9. https://doi.org/10.1093/jnen/61.3.215

  12. Liberati A, Altman DG, Tetzlaff J, Mulrow C, Gøtzsche PC, Ioannidis JP, Clarke M, Devereaux PJ, Kleijnen J, Moher D (2009) The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate health care interventions: explanation and elaboration. PLoS Med 6:e1000100. https://doi.org/10.1371/journal.pmed.1000100

    Article  PubMed  PubMed Central  Google Scholar 

  13. Alharbi A, Alkhaibary A, Alaglan A, Khairy S, Alkhunaizi Z, AlSufiani F, Alassiri AH, Alkhani A (2022) Sellar xanthogranuloma: a diagnostic challenge. Surg Neurol Int 13:76. https://doi.org/10.25259/SNI_1180_2021

  14. Ayadi K, Gallaoui S, Ben Nsir A (2021) Pituitary apoplexy, but not as we thought: a xanthogranuloma rather than an adenoma. Lancet 398(10311):e16. https://doi.org/10.1016/S0140-6736(21)01898-5

    Article  PubMed  Google Scholar 

  15. Cheng D, Yang F, Li Z, Qv F, Liu W (2021) Juvenile xanthogranuloma of the sellar region with a 5-year medical history: case report and literature review. Pediatr Neurosurg 56:440–447. https://doi.org/10.1159/000515517

    Article  PubMed  Google Scholar 

  16. Shao X, Wang C, Min J (2020) Xanthogranuloma of the sellar region: a case report. Medicine (Baltimore) 99:e22619. https://doi.org/10.1097/MD.0000000000022619

    Article  PubMed  Google Scholar 

  17. Pilonieta M, Martin M, Revuelta Barbero JM, Hardesty DA, Carrau RL, Otto BA, Otero J, Ghalib L, Prevedello DM (2020) Sellar cholesterol granuloma mimicking cystic sellar lesions: a report of three cases and literature review. World Neurosurg 144:250–255. https://doi.org/10.1016/j.wneu.2020.07.234

    Article  PubMed  Google Scholar 

  18. Vasquez CA, Downes A, Kleinschmidt-DeMasters BK, Youssef AS (2019) Functioning pituitary adenoma with xanthogranulomatous features: review of literature and case report. J Neurol Surg B Skull Base 80:449–457. https://doi.org/10.1055/s-0038-1675232

    Article  PubMed  Google Scholar 

  19. Stojanovic M, Manojlovic-Gacic E, Pekic S, Milojevic T, Miljic D, Doknic M, Nikolic Djurovic M, Jemuovic Z, Petakov M (2019) From diabetes insipidus to sellar xanthogranuloma - a “yellow brick road” demanding team-work. Acta Endocrinol (Buchar) 15:247–253. https://doi.org/10.4183/aeb.2019.247

    Article  CAS  PubMed  Google Scholar 

  20. Fujio S, Takajo T, Kinoshita Y, Hanaya R, Arimura H, Sugata J, Sugata S, Bohara M, Hiraki T, Yoshimoto K, Arita K (2019) Sellar xanthogranuloma: a quest based on nine cases assessed with an anterior pituitary provocation test. World Neurosurg 130:e150–e159. https://doi.org/10.1016/j.wneu.2019.06.021

    Article  PubMed  Google Scholar 

  21. Cho SM, Cho HR, Park YS, Chang HG (2018) Giant sellar xanthogranuloma after surgical treatment of symptomatic Rathke’s cleft cyst. Brain Tumor Res Treat 6:82–85. https://doi.org/10.14791/btrt.2018.6.e10

    Article  PubMed  PubMed Central  Google Scholar 

  22. La Rocca G, Rigante M, Gessi M, D’Alessandris QG, Auricchio AM, Chiloiro S, De Marinis L, Lauretti L (2019) Xanthogranuloma of the sellar region: a rare tumor. Case illustration and literature review. J Clin Neurosci 59:318–324. https://doi.org/10.1016/j.jocn.2018.10.019

    Article  PubMed  Google Scholar 

  23. Kobayashi M, Yagasaki H, Kobayashi K, Ogiwara M, Kinouchi H, Sugita K (2018) Xanthogranuloma of the sellar region accompanied by growth hormone deficiency: case report and literature review. J Pediatr Endocrinol Metab 31:1161–1164. https://doi.org/10.1515/jpem-2018-0050

    Article  PubMed  Google Scholar 

  24. Nishimura F, Park YS, Motoyama Y, Nakagawa I, Yamada S, Nakase H (2018) Pediatric case of xanthogranuloma in the sellar region presenting a visual disturbance successfully treated with endoscopic endonasal surgery. World Neurosurg 114:22–26. https://doi.org/10.1016/j.wneu.2018.02.155

    Article  PubMed  Google Scholar 

  25. Ved R, Logier N, Leach P, Davies JS, Hayhurst C (2018) Pituitary xanthogranulomas: clinical features, radiological appearances and post-operative outcomes. Pituitary 21:256–265. https://doi.org/10.1007/s11102-017-0859-x

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  26. Ji K, Zhang L, Wang L, Wang W (2016) Xanthogranuloma of the sellar region diagnosed by frozen section. Open Med (Wars) 11:426–428. https://doi.org/10.1515/med-2016-0076

    Article  PubMed  Google Scholar 

  27. Céspedes MT, Vargas JP, Yañez FA, León LS, Arancibia PÁ, Putz TS (2017) Remarkable diagnostic magnetic resonance imaging findings in sellar xanthogranuloma: report of three first cases in Latin America. J Neurol Surg Rep 78:e26–e33. https://doi.org/10.1055/s-0037-1598203

    Article  PubMed  PubMed Central  Google Scholar 

  28. Mohanty S, Mohanty L, Bano S, Pattnaik K (2017) Xanthogranuloma (xg) masquerading as tumour of sellar region- a diagnostic challenge. Evid Based Med Healthc 4:4567–4570. https://doi.org/10.18410/jebmb/2017/912

    Article  Google Scholar 

  29. Dai CX, Guo XS, Liu XH, Bao XJ, Feng M, Zhong DR, Ma WB, Wang RZ, Yao Y (2017) Xanthogranuloma of the sellar region. Chin Med J (Engl) 130:249–250. https://doi.org/10.4103/0366-6999

    Article  PubMed  Google Scholar 

  30. Hernández-Estrada RA, Kshettry VR, Vogel AN, Curtis MT, Evans JJ (2017) Cholesterol granulomas presenting as sellar masses: a similar, but clinically distinct entity from craniopharyngioma and Rathke’s cleft cyst. Pituitary 20:325–332. https://doi.org/10.1007/s11102-016-0775-5

    Article  PubMed  Google Scholar 

  31. Gurcay AG, Gurcan O, Kazanci A, Bozkurt I, Senturk S, Ferat M, Turkoglu OF, Beskonakli E, Orhun Yavuz HS (2016) Xanthogranuloma of the sellar region. Neurol India 64:1075–1079. https://doi.org/10.4103/0028-3886

    Article  PubMed  Google Scholar 

  32. Petrakakis I, Pirayesh A, Krauss JK, Raab P, Hartmann C, Nakamura M (2016) The sellar and suprasellar region: a “hideaway” of rare lesions. Clinical aspects, imaging findings, surgical outcome and comparative analysis. Clin Neurol Neurosurg 149:154–165. https://doi.org/10.1016/j.clineuro.2016.08.011

    Article  PubMed  Google Scholar 

  33. Ben Nsir A, Thai QA, Chaieb L, Jemel H (2015) Calcified suprasellar xanthogranuloma presenting with primary amenorrhea in a 17-year-old girl: case report and literature review. World Neurosurg 84:866.e11-866.e8.66E14. https://doi.org/10.1016/j.wneu.2015.04.024

    Article  PubMed  Google Scholar 

  34. Rahmani R, Sukumaran M, Donaldson AM, Akselrod O, Lavi E, Schwartz TH (2015) Parasellar xanthogranulomas. J Neurosurg 122:812–817. https://doi.org/10.3171/2014.12.JNS14542

    Article  PubMed  Google Scholar 

  35. Madan Mohan B, Mohamed E, Jain SK, Jain M, Jaiswal AK (2015) Serial MR imaging in suprasellar xanthogranuloma: growth pattern and new lesions. J Neuroimaging 25:677–679. https://doi.org/10.1111/jon.12170

    Article  PubMed  Google Scholar 

  36. Amano K, Kubo O, Komori T, Tanaka M, Kawamata T, Hori T, Okada Y (2013) Clinicopathological features of sellar region xanthogranuloma: correlation with Rathke’s cleft cyst. Brain Tumor Pathol 30:233–241. https://doi.org/10.1007/s10014-012-0130-0

    Article  PubMed  Google Scholar 

  37. Agarwal A, Agarwal K, Lee HK (2012) Xanthogranuloma of the sellar region a case report. Neuroradiol J 25:181–184. https://doi.org/10.1177/197140091202500204

    Article  CAS  PubMed  Google Scholar 

  38. Nishiuchi T, Murao K, Imachi H, Kushida Y, Haba R, Kawai N, Tamiya T, Ishida T (2012) Xanthogranuloma of the intrasellar region presenting in pituitary dysfunction: a case report. J Med Case Rep 9(6):119. https://doi.org/10.1186/1752-1947-6-119

    Article  Google Scholar 

  39. Tsai ST, Tsui YK, Wu MWL, Chen TY, Tzeng WS (2012) Xanthogranuloma of sellar region: a case report with diagnostic imaging findings, treatment response and literature review. J Radiol Sci 37:89–94

    Google Scholar 

  40. Kamoshima Y, Sawamura Y, Motegi H, Kubota K, Houkin K (2011) Xanthogranuloma of the sellar region of children: series of five cases and literature review. Neurol Med Chir (Tokyo) 51:689–693. https://doi.org/10.2176/nmc.51.689

    Article  PubMed  Google Scholar 

  41. Sulentić P, Cupić H, Cerina V, Vrkljan M (2010) Xanthogranuloma of the sellar region in a patient with sarcoidosis. Acta Clin Croat 49:61–65

    PubMed  Google Scholar 

  42. Arai A, Nishihara M, Sasayama T, Aihara H, Hosoda K, Itoh T, Sakagami Y, Kuwamura K, Kohmura E (2010) Xanthogranuloma of the sellar region–case report. Neurol Med Chir (Tokyo) 50:488–491. https://doi.org/10.2176/nmc.50.488

    Article  PubMed  Google Scholar 

  43. Sugata S, Hirano H, Yatsushiro K, Yunoue S, Nakamura K, Arita K (2009) Xanthogranuloma in the suprasellar region. Neurol Med Chir (Tokyo) 49:124–127. https://doi.org/10.2176/nmc.49.124

    Article  PubMed  Google Scholar 

  44. Liu ZH, Tzaan WC, Wu YY, Chen HC (2008) Sellar xanthogranuloma manifesting as obstructive hydrocephalus. J Clin Neurosci 15:929–933. https://doi.org/10.1016/j.jocn.2007.05.028

    Article  PubMed  Google Scholar 

  45. Pavón de Paz I, Peñalver Talavera D, Olivar Roldán J, Carlos Gómez-Angulo Giner J, Santonja Garriga C, Monereo Megías S (2008) Sellar xanthogranuloma. Endocrinol Nutr 55:102–106. https://doi.org/10.1016/S1575-0922(08)70644-2

    Article  PubMed  Google Scholar 

  46. Tajima T, Sawamura Y, Ishizu K, Tsubaki J (2006) Two children with xanthogranuloma of the sellar region. Clin Pediatr Endocrinol 15:85–91. https://doi.org/10.1297/cpe.15.85

    Article  PubMed  PubMed Central  Google Scholar 

  47. Jung CS, Schänzer A, Hattingen E, Plate KH, Seifert V (2006) Xanthogranuloma of the sellar region. Acta Neurochir (Wien) 148:473–477. https://doi.org/10.1007/s00701-005-0700-6

    Article  CAS  PubMed  Google Scholar 

  48. Burt MG, Morey AL, Turner JJ, Pell M, Sheehy JP, Ho KK (2003) Xanthomatous pituitary lesions: a report of two cases and review of the literature. Pituitary 6:161–168. https://doi.org/10.1023/b:pitu.0000011177.43408.56

    Article  PubMed  Google Scholar 

  49. Yonezawa K, Shirataki K, Sakagami Y, Kohmura E (2003) Panhypopituitarism induced by cholesterol granuloma in the sellar region-case report. Neurol Med Chir (Tokyo) 43:259–262. https://doi.org/10.2176/nmc.43.259

    Article  PubMed  Google Scholar 

  50. Le BH, Towfighi J, Kapadia SB, Lopes MB (2007) Comparative immunohistochemical assessment of craniopharyngioma and related lesions. Endocr Pathol 18:23–30. https://doi.org/10.1007/s12022-007-0011-y

    Article  CAS  PubMed  Google Scholar 

  51. Duan K, Asa SL, Winer D, Gelareh Z, Gentili F, Mete O (2017) Xanthomatous hypophysitis is associated with ruptured Rathke’s cleft cyst. Endocr Pathol 28:83–90. https://doi.org/10.1007/s12022-017-9471-x

    Article  PubMed  Google Scholar 

  52. Nishioka H, Shibuya M, Ohtsuka K, Ikeda Y, Haraoka J (2010) Endocrinological and MRI features of pituitary adenomas with marked xanthogranulomatous reaction. Neuroradiology 52:997–1002. https://doi.org/10.1007/s00234-010-0675-8

    Article  PubMed  Google Scholar 

  53. Türe U, De Bellis A, Harput MV, Bellastella G, Topcuoglu M, Yaltirik CK, Cirillo P, Yola RN, Sav A, Kelestimur F (2021) Hypothalamitis: a novel autoimmune endocrine disease. A literature review and case report. J Clin Endocrinol Metab 106(2):e415–e429. https://doi.org/10.1210/clinem/dgaa771

    Article  PubMed  Google Scholar 

  54. Yang B, Yang C, Fang J, Li G, Wang J, Jia G, Liu P, Jia W, Wang S (2017) Clinicoradiologic features and surgical outcomes of sellar xanthogranulomas: a single-center 10-year experience. World Neurosurg 99:439–447. https://doi.org/10.1016/j.wneu.2016.12.017

    Article  PubMed  Google Scholar 

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Authors and Affiliations

  1. Department of Endocrinology, Bellvitge University Hospital-Institut d’Investigació Biomèdica de Bellvitge (IDIBELL), Carrer de la Feixa Llarga, s/n, L’Hospitalet de Llobregat, 08907, Barcelona, Spain

    Fernando Guerrero-Pérez, Nuria Vilarrasa, Manuel Pérez-Maraver & Carles Villabona

  2. Department of Endocrinology, CIMA-Sanitas Hospital, Barcelona, Spain

    Agustina Pia Marengo

  3. CIBERDEM-CIBER de Diabetes y Enfermedades Metabólicas Asociadas, Instituto de Salud Carlos III, Barcelona, Spain

    Nuria Vilarrasa & Manuel Pérez-Maraver

  4. Department of Pathology, Bellvitge University Hospital-IDIBELL, L’Hospitalet de Llobregat, Barcelona, Spain

    Noemi Vidal & Nuria Ruiz-Roig

  5. Neuro-Oncology Unit-IDIBELL, L’Hospitalet de Llobregat, Barcelona, Spain

    Noemi Vidal

  6. Institut de Diagnòstic per la Imatge, Bellvitge University Hospital-IDIBELL, L’Hospitalet de Llobregat, Barcelona, Spain

    Juan José Sánchez-Fernández

  7. Department of Neurosurgery, Bellvitge University Hospital-IDIBELL, L’Hospitalet de Llobregat, Barcelona, Spain

    Alberto Torres-Díaz & José Luis Sanmillán

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  1. Fernando Guerrero-Pérez
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Contributions

FG-P and CV (conceived the study and wrote original draft); FG-P, APM, and NV (acquisition and curation of data); FG-P, APM, NV, and NRR (analysis and interpretation of data); and JJSF, JLS, ATD, and MPM (critical revision of manuscript). All authors have read and agreed to publish the final version of the manuscript.

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Correspondence to Fernando Guerrero-Pérez.

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Guerrero-Pérez, F., Marengo, A.P., Vilarrasa, N. et al. Xanthogranuloma of the sellar region: a systematic review. Hormones 22, 199–210 (2023). https://doi.org/10.1007/s42000-023-00432-y

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