Craniopharyngioma Explained

Pituicytoma

A craniopharyngioma is a rare type of brain tumor derived from pituitary gland embryonic tissue[1] that occurs most commonly in children, but also affects adults. It may present at any age, even in the prenatal and neonatal periods, but peak incidence rates are childhood-onset at 5–14 years and adult-onset at 50–74 years.[2] People may present with bitemporal inferior quadrantanopia leading to bitemporal hemianopsia, as the tumor may compress the optic chiasm. It has a point prevalence around two per 1,000,000.[3] Craniopharyngiomas are distinct from Rathke's cleft tumours and intrasellar arachnoid cysts.[4]

Symptoms and signs

Craniopharyngiomas are almost always benign.[5] However, as with many brain tumors, their treatment can be difficult, and significant morbidities are associated with both the tumor and treatment.

Mechanisms

Craniopharyngioma is a rare, usually suprasellar[15] neoplasm, which may be cystic, that develops from nests of epithelium derived from Rathke's pouch.[16] [17] Rathke's pouch is an embryonic precursor of the anterior pituitary.

Craniopharyngiomas are typically very slow-growing tumors. They arise from the cells along the pituitary stalk, specifically from nests of odontogenic (tooth-forming) epithelium within the suprasellar/diencephalic region, so contain deposits of calcium that are evident on an X-ray.[18]

Diagnosis

Imaging scans for craniopharyngioma

A physician can conduct a few scans and tests to diagnose a person with craniopharyngioma.[19] High-resolution magnetic resonance imaging (MRI) is commonly used as a diagnostic tool; however, computer tomography (CT) remains the gold standard imaging choice for craniopharyngioma diagnosis as it can detect the severity of the calcification within the tumour.[20]

In some cases, a powerful 3T (Tesla) MRI scanner can help define the location of critical brain structures affected by the tumor. The histologic pattern consists of nesting of squamous epithelium bordered by radially arranged cells. It is frequently accompanied by calcium deposition and may have a microscopic papillary architecture. A computed tomography (CT) scan is also a good diagnostic tool, as it detects calcification in the tumor.[21]

Two distinct types are recognized:[22] [23]

In the adamantinomatous type, calcifications are visible on neuroimaging and are helpful in diagnosis.

The papillary type rarely calcifies. A vast majority of craniopharyngiomas in children are adamantinomatous, whereas both subtypes are common in adults. Mixed-type tumors also occur.[25]

On macroscopic examination, craniopharyngiomas are cystic or partially cystic with solid areas. On light microscopy, the cysts are seen to be lined by stratified squamous epithelium. Keratin pearls may also be seen. The cysts are usually filled with a yellow, viscous fluid rich in cholesterol crystals. Of a long list of possible symptoms, the most common presentations include headaches, growth failure, and bitemporal hemianopsia.

Malignant craniopharyngioma

Craniopharyngiomas are usually successfully managed with a combination of adjuvant chemotherapy and neurosurgery. Recent research describes the rare occurrence of malignant transformations of these normally benign tumors. Malignant craniopharyngiomas can occur at any age, are slightly more common in females, and are usually of the adamantinomatous type.[26]

The malignant transformations can take years to occur (although one in five of the diagnosed cases were de novo transformations), hence the need for lengthier follow-up in patients diagnosed with the more common benign forms.

No link has been found between malignancy and initial chemoradiotherapy treatment, and the overall survival rate was very poor with median survival being 6 months after diagnosis of malignancy.

Prevention

Although the causes of craniopharyngioma are unknown, it can occur in both children and adults, with a peak in incidence at 9 to 14 years of age. About 120 cases are diagnosed each year in the United States in patients under the age of 19. More than 50% of all patients with craniopharyngioma are under the age of 18 years. No clear association of the tumor exists with a particular gender or race. Craniopharyngiomas do not appear to "run in families" or to be directly inherited from the parents.

Treatment

Treatment generally consists of subfrontal or transsphenoidal excision. Endoscopic surgery through the nose[27] [28] often performed by a team of neurosurgeons and ENT, is an alternative to transcranial surgery done by making an opening in the skull. Because of the location of the craniopharyngioma in the skull base of the brain, a surgical navigation system might be used to verify the position of surgical tools during the operation.[29]

Additional radiotherapy is used if total removal is not possible. Due to the poor outcomes associated with damage to the pituitary and hypothalamus from surgical removal and radiation, experimental therapies using intracavitary phosphorus-32, yttrium, or bleomycin delivered via an external reservoir are sometimes employed, especially in young patients. The tumor, being in the pituitary gland, can cause secondary health problems. The immune system, thyroid levels, growth hormone levels, and testosterone levels can be compromised from craniopharyngioma. All of these health problems can be treated with modern medicine.[30] No high quality evidence has evaluated the use of bleomycin in this condition.[31]

Proton therapy affords a reduction in dose to critical structures compared to conventional photon radiation, including IMRT, for patients with craniopharyngioma.[32]

The most effective treatment 'package' for the malignant craniopharyngiomas described in literature is a combination 'gross total resective' surgery with adjuvant chemoradiotherapy. The chemotherapy drugs paclitaxel and carboplatin have shown a clinical (but not statistical) significance in increasing the survival rate in patients who have had gross total resections of their malignant tumours.

Prognosis

Craniopharyngiomas are generally benign but are known to recur after resection. Recent research has demonstrated a malignant (but rare) tendency of craniopharyngiomas. These malignant craniopharyngiomas are very rare but are associated with poor prognosis.

Recent research

Current [as of when?] research has shown ways of treating the tumors in a less invasive way while others have shown how the hypothalamus can be stimulated along with the tumor to prevent the child and adult with the tumor to become obese. A childhood craniopharyngioma is commonly cystic in nature.[33] Limited surgery minimizing hypothalamic damage may decrease the severe obesity rate at the expense of the need for radiotherapy to complete the treatment.[34]

Role of radiotherapy:

Aggressive attempts at total removal do result in prolonged progression-free survival in most patients, but for tumors that clearly involve the hypothalamus, complications associated with radical surgery have prompted to adopt a combined strategy of conservative surgery and radiation therapy to residual tumor with as high a rate of cure. This strategy seems to offer the best long-term control rates with acceptable morbidity. But optimal management of craniopharyngiomas remains controversial. Although it is generally recommended that radiotherapy is given following subtotal excision of a craniopharyngioma, it remains unclear as to whether all patients with residual tumour should receive immediate or differed at relapse radiotherapy.[35] Surgery and radiotherapy are the cornerstones in therapeutic management of craniopharyngioma. Radical excision is associated with a risk of mortality or morbidity particularly as hypothalamic damage, visual deterioration, and endocrine complication between 45 and 90% of cases. The close proximity to neighboring eloquent structures poses a particular challenge to radiation therapy. Modern treatment technologies including fractionated 3-D conformal radiotherapy,[36] intensity modulated radiation therapy, and recently proton therapy are able to precisely cover the target while preserving surrounding tissue, Tumor controls in excess of 90% can be achieved. Alternative treatments consisting of radiosurgery, intracavitary application of isotopes, and brachytherapy also offer an acceptable tumor control and might be given in selected cases. More research is needed to establish the role of each treatment modality.[37]

External links

Notes and References

  1. Web site: Craniopharyngioma. The Lecturio Medical Concept Library . 10 July 2021.
  2. Müller HL . Craniopharyngioma . Endocrine Reviews . 35 . 3 . 513–43 . June 2014 . 24467716 . 10.1210/er.2013-1115 . free .
  3. Garnett MR, Puget S, Grill J, Sainte-Rose C . Craniopharyngioma . Orphanet Journal of Rare Diseases . 2 . 18 . April 2007 . 17425791 . 1855047 . 10.1186/1750-1172-2-18 . free .
  4. Shin JL, Asa SL, Woodhouse LJ, Smyth HS, Ezzat S . Cystic lesions of the pituitary: clinicopathological features distinguishing craniopharyngioma, Rathke's cleft cyst, and arachnoid cyst . The Journal of Clinical Endocrinology and Metabolism . 84 . 11 . 3972–82 . November 1999 . 10566636 . 10.1210/jcem.84.11.6114 . free .
  5. Garrè ML, Cama A . Craniopharyngioma: modern concepts in pathogenesis and treatment . Current Opinion in Pediatrics . 19 . 4 . 471–479 . August 2007 . 17630614 . 10.1097/MOP.0b013e3282495a22 . 25955091 .
  6. Web site: Hydrocephalus Fact Sheet . April 5, 2016 . NINDS . https://web.archive.org/web/20160727231854/http://www.ninds.nih.gov/disorders/hydrocephalus/detail_hydrocephalus.htm . 27 July 2016 . live . 5 September 2016.
  7. Reynolds CF, O'Hara R . DSM-5 sleep-wake disorders classification: overview for use in clinical practice . The American Journal of Psychiatry . 170 . 10 . 1099–1101 . October 2013 . 24084814 . 10.1176/appi.ajp.2013.13010058 . The aim is simply to acknowledge the bidirectional and interactive effects between sleep disorders and coexisting medical and psychiatric illnesses. .
  8. Ahmet A, Blaser S, Stephens D, Guger S, Rutkas JT, Hamilton J . Weight gain in craniopharyngioma--a model for hypothalamic obesity . Journal of Pediatric Endocrinology & Metabolism . 19 . 2 . 121–127 . February 2006 . 16562584 . 10.1515/jpem.2006.19.2.121 . 35451441 .
  9. Book: Porth CM . 1990 . Pathophysiology: Concepts of altered health states . Philadelphia . Philadelphia: J.B. Lippincott Company .
  10. Web site: Diabetes Insipidus. The Lecturio Medical Concept Library . 26 October 2020. 11 July 2021.
  11. News: Bitemporal hemianopsia. Science Daily.
  12. Web site: Pediatric Vomiting. The Lecturio Medical Concept Library . 19 July 2021.
  13. Web site: Craniopharyngioma UCLA Pituitary Tumor Program. pituitary.ucla.edu. 2017-12-09.
  14. Web site: Diabetes insipidus - Symptoms and causes - Mayo Clinic. www.mayoclinic.org. en. 2017-12-09.
  15. Rodriguez FJ, Scheithauer BW, Tsunoda S, Kovacs K, Vidal S, Piepgras DG . The spectrum of malignancy in craniopharyngioma . The American Journal of Surgical Pathology . 31 . 7 . 1020–1028 . July 2007 . 17592268 . 10.1097/PAS.0b013e31802d8a96 . 36172034 .
  16. Book: Moore KD, Couldwell WT . William T. Couldwell --> . Bernstein M, Berger MS . Joint Tumor Section of the American Association of Neurological Surgeons & the Congress of Neurological Surgeons . Neuro-oncology: the essentials . August 8, 2011 . January 15, 2000 . Thieme . 978-0-86577-880-1 . 409–418 . 41. Craniopharyngioma . https://books.google.com/books?id=gbwSC43Eia4C&pg=PA409 .
  17. Web site: Craniopharyngioma . Organ System Pathology Images, The Internet Pathology Laboratory for Medical Education . The University of Utah Eccles Health Sciences Library . 2009-05-08.
  18. Web site: Craniopharyngioma. The Lecturio Medical Concept Library . 22 July 2021.
  19. News: Craniopharyngioma - Childhood: Diagnosis Cancer.Net. 2012-06-25. Cancer.Net. 2017-12-09. en.
  20. Müller . Hermann L. . 2020 . The Diagnosis and Treatment of Craniopharyngioma . Neuroendocrinology . english . 110 . 9–10 . 753–766 . 10.1159/000504512 . 0028-3835 . 31678973. 207904477 . free .
  21. Web site: Craniopharyngioma. UCLA Health.
  22. Sekine S, Shibata T, Kokubu A, Morishita Y, Noguchi M, Nakanishi Y, Sakamoto M, Hirohashi S . 6 . Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations . The American Journal of Pathology . 161 . 6 . 1997–2001 . December 2002 . 12466115 . 1850925 . 10.1016/s0002-9440(10)64477-x .
  23. Sekine S, Takata T, Shibata T, Mori M, Morishita Y, Noguchi M, Uchida T, Kanai Y, Hirohashi S . 6 . Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma: evidence for its odontogenic epithelial differentiation . Histopathology . 45 . 6 . 573–579 . December 2004 . 15569047 . 10.1111/j.1365-2559.2004.02029.x . 35192721 .
  24. Brastianos PK, Taylor-Weiner A, Manley PE, Jones RT, Dias-Santagata D, Thorner AR, Lawrence MS, Rodriguez FJ, Bernardo LA, Schubert L, Sunkavalli A, Shillingford N, Calicchio ML, Lidov HG, Taha H, Martinez-Lage M, Santi M, Storm PB, Lee JY, Palmer JN, Adappa ND, Scott RM, Dunn IF, Laws ER, Stewart C, Ligon KL, Hoang MP, Van Hummelen P, Hahn WC, Louis DN, Resnick AC, Kieran MW, Getz G, Santagata S . 6 . Exome sequencing identifies BRAF mutations in papillary craniopharyngiomas . Nature Genetics . 46 . 2 . 161–165 . February 2014 . 24413733 . 3982316 . 10.1038/ng.2868 .
  25. Weiner HL, Wisoff JH, Rosenberg ME, Kupersmith MJ, Cohen H, Zagzag D, Shiminski-Maher T, Flamm ES, Epstein FJ, Miller DC . 6 . Craniopharyngiomas: a clinicopathological analysis of factors predictive of recurrence and functional outcome . Neurosurgery . 35 . 6 . 1001–10; discussion 1010–1 . December 1994 . 7885544 . 10.1227/00006123-199412000-00001 .
  26. Sofela AA, Hettige S, Curran O, Bassi S . Malignant transformation in craniopharyngiomas . Neurosurgery . 75 . 3 . 306–14; discussion 314 . September 2014 . 24978859 . 10.1227/NEU.0000000000000380 . 44735428 .
  27. Dhandapani S, Singh H, Negm HM, Cohen S, Souweidane MM, Greenfield JP, Anand VK, Schwartz TH . 6 . Endonasal endoscopic reoperation for residual or recurrent craniopharyngiomas . Journal of Neurosurgery . 126 . 2 . 418–430 . February 2017 . 27153172 . 10.3171/2016.1.JNS152238 . free .
  28. Web site: Endoscopic pituitary surgery (transsphenoidal) . Mayfield Brain & Spine . Mayfield Clinic .
  29. Web site: Use of Surgical Navigation for Craniopharyngioma Removal. January 2013.
  30. Wisoff JH . Craniopharyngioma . Journal of Neurosurgery. Pediatrics . 1 . 2 . 124–5; discussion 125 . February 2008 . 18352780 . 10.3171/PED/2008/1/2/124 .
  31. Zhang S, Fang Y, Cai BW, Xu JG, You C . Intracystic bleomycin for cystic craniopharyngiomas in children . The Cochrane Database of Systematic Reviews . 2016 . CD008890 . July 2016 . 7 . 27416004 . 6457977 . 10.1002/14651858.CD008890.pub4 .
  32. Web site: Treating Pediatric Tumors With Proton Therapy Current Practice, Opportunities and Challenges. IBA. October 2015. 2018-05-03. 2019-04-02. https://web.archive.org/web/20190402084627/https://iba-worldwide.com/download/iba-white-paper-treating-pediatric-tumors-with-proton-therapy. dead.
  33. Roth C, Wilken B, Hanefeld F, Schröter W, Leonhardt U . Hyperphagia in children with craniopharyngioma is associated with hyperleptinaemia and a failure in the downregulation of appetite . European Journal of Endocrinology . 138 . 1 . 89–91 . January 1998 . 9461323 . 10.1530/eje.0.1380089 . free .
  34. Elowe-Gruau E, Beltrand J, Brauner R, Pinto G, Samara-Boustani D, Thalassinos C, Busiah K, Laborde K, Boddaert N, Zerah M, Alapetite C, Grill J, Touraine P, Sainte-Rose C, Polak M, Puget S . 6 . Childhood craniopharyngioma: hypothalamus-sparing surgery decreases the risk of obesity . The Journal of Clinical Endocrinology and Metabolism . 98 . 6 . 2376–2382 . June 2013 . 23633208 . 10.1210/jc.2012-3928 . free .
  35. Chargari C, Bauduceau O, Bauduceau B, Camparo P, Ceccaldi B, Fayolle M, Le Moulec S, Védrine L . 6 . [Craniopharyngiomas: role of radiotherapy] ]. Bulletin du Cancer . 94 . 11 . 987–994 . November 2007 . 18055317 .
  36. Web site: Three Dimensional (3D) Conformal Radiation Therapy . hillman.upmc.com. en. 2017-12-09.
  37. Kortmann RD . Different approaches in radiation therapy of craniopharyngioma . Frontiers in Endocrinology . 2 . 100 . 2011-12-20 . 22654836 . 3356005 . 10.3389/fendo.2011.00100 . free .

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