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Craniopharyngioma

Craniopharyngiomas

Craniopharyngioma (Erdheim tumor) is a rare, benign neoplasm, that develops from epithelium derived from Rathke's pouch and usually affects children.


Presentation

This tumor-type is one that exhibits slow growth. The symptoms often show insidiously and they do not become obvious until the tumor has attained a diameter of about 3cm [7]. The interval between the first symptoms and actual diagnosis often ranges between 1-2 years.

The most common symptoms are as follows:

The headache is dull, continuous and positional. It is equally progressive and becomes severe when the endocrine symptoms have become obvious [8].

Again, 40% of patients exhibit constipation, cold intolerance, fatigue and weight gain. All of these are signs of hypothyroidism.
25% of patients have equally shown vomiting, nausea, anorexia, confusion, lethargy, cardiac arrhythmias, hyperkalemia, hypoglycaemia and orthostatic hypotension. All of these are signs of adrenal failure.

20% of patients have also shown presence of diabetes insipidus characterised by excessive fluid intake and urination. In younger patients, delayed puberty and growth failure are other symptoms.
80% of adults complain of decrease in sexual drive while 90% of men show impotence. Most women equally complain about amenorrhea.

Hypothermia
  • Impaired thermoregulation with hyper- or hypothermia has also been observed in adults (Lipton et al., 1981 ; Griffiths et al., 1988 ).[ncbi.nlm.nih.gov]
Vomiting
  • An 8-year-old boy presented complaining principally of headache and vomiting.[ncbi.nlm.nih.gov]
  • A 21-year-old man presented to the authors' service with a 3-day history of worsening headache, nausea, vomiting, and blurry vision.[ncbi.nlm.nih.gov]
  • The onset of his illness had been preceded by vomiting and diarrhea for 1 day which he attributed to food poisoning. On examination, he had an apathetic disposition with a generalized "sallow complexion." He was not dehydrated.[ncbi.nlm.nih.gov]
  • Common symptoms include: Headache Nausea Vomiting Personality changes Confusion Difficulty with balance Growth failure[symptoma.com]
  • As it grows, the tumour may compress the optic nerve and other nearby structures, causing loss of vision , headaches , vomiting , behavioral changes, endocrine disorders, and sometimes loss of the sense of smell .[britannica.com]
Nausea
  • A 21-year-old man presented to the authors' service with a 3-day history of worsening headache, nausea, vomiting, and blurry vision.[ncbi.nlm.nih.gov]
  • A 52-year-old Sri Lankan man presented with anorexia, nausea, fatigue, generalized muscle weakness, and cramps for 1 week. The onset of his illness had been preceded by vomiting and diarrhea for 1 day which he attributed to food poisoning.[ncbi.nlm.nih.gov]
  • Common symptoms include: Headache Nausea Vomiting Personality changes Confusion Difficulty with balance Growth failure[symptoma.com]
  • Call your provider for the following symptoms: Headache, nausea, vomiting, or balance problems (signs of increased pressure on the brain) Increased thirst and urination Poor growth in a child Vision changes Ater JL, Kuttesch JF.[medlineplus.gov]
Loss of Peripheral Vision
  • […] of peripheral vision Excessive thirst Increased urination Slow or halted growth Excessive weight gain Early or delayed puberty If you are concerned about any changes your child experiences, please talk with your child’s doctor.[cancer.net]
  • The most common symptoms of this tumor are visual changes, most often loss of peripheral vision in one eye (visual field cuts) or blurriness. This is because of the proximity of craniopharyngiomas to the visual nerve pathways in the brain.[oncolink.org]
Visual Field Constriction
  • The authors present the case of a 12-year-old boy who developed progressive visual field constriction 11 years after gross-total resection of a solid and cystic craniopharyngioma.[ncbi.nlm.nih.gov]
Headache
  • A 13-year-old boy presented with headache for 2 years after resection of craniopharyngioma. A fusiform dilation of the right carotid artery was found and was coiled using stent-assisted technique.[ncbi.nlm.nih.gov]
  • Clinicians need to be aware of this association, and should perform urgent MRI scanning in TS patients with headache, visual impairment or clinical/biochemical evidence of hypopituitarism.[ncbi.nlm.nih.gov]
  • Typically, craniopharyngioma were of adamantinomatous type, occurred simultaneously to pituitary adenoma, presented with headache and visual loss, and affected men.[ncbi.nlm.nih.gov]
  • When RCCs become symptomatic in children, the most common symptom they lead to is headache. The cysts are commonly small, regular, and oval in shape.[ncbi.nlm.nih.gov]
  • This 31-year-old man presented with headache and dizziness. Brain CT and MRI showed a 5 4-cm lesion with multiple small calcifications in the left CPA. The patient underwent suboccipital craniotomy with tumor removal.[ncbi.nlm.nih.gov]
Bitemporal Hemianopsia
  • Of the seventeen (63%) patients presenting with varying degrees of bitemporal hemianopsia, all had improvement in vision postoperatively. It is worth noting that no cases of preoperative hypopituitarism or DI improved postoperatively.[ncbi.nlm.nih.gov]
  • This is known as “bitemporal hemianopsia,” and is a result of the tumor pressing on the optic chiasm (a structure where the two optic nerves cross). As the tumor enlarges it can lead to blindness in one or both eyes.[neurosurgery.ufl.edu]
  • Bitemporal hemianopsia (missing vision in the periphery of the right and left visual fields due to pressure on the optic chiasm), Double vision Endocrine abnormalities: Hypothyroidism (40% of cases): Weight gain Fatigue Cold intolerance Constipation Nonpitting[pedclerk.bsd.uchicago.edu]
  • Case Report A 73-year-old man initially presented in March 1990 with symptoms of bitemporal hemianopsia.[ajnr.org]
Amenorrhea
  • A 30-year-old woman presented with a 5-month history of amenorrhea and was admitted to the hospital. She underwent surgical resection for three times and died at last. MRI revealed a new solid component of craniopharyngioma.[ncbi.nlm.nih.gov]
  • […] features of pituitary adenoma presenting in association with craniopharyngioma; (2) to describe the first documented (clinically, biochemically, histologically, and radiologically) case of aggressive, suprasellar papillary craniopharyngioma presenting with amenorrhea[ncbi.nlm.nih.gov]
  • Most women equally complain about amenorrhea.[symptoma.com]
  • Development of the tumor after puberty usually results in amenorrhea in women and loss of libido and potency in men. Also called ameloblastoma, craniopharyngeal duct tumor, pituitary adamantinoma, Rathke's pouch tumor .[medical-dictionary.thefreedictionary.com]
  • […] medulloblastoma) Histoligically benign tumors Clinically present with headaches ( 50%), visual disturbances (40-70%), and pituitary dysfunction (up to 90%) Children: hypothyroid and GH deficiency Adults: sexual dysfunction (men erectile dysfunction, women amenorrhea[en.wikibooks.org]
Sexual Dysfunction
  • Pituitary dysfunction is also common; children may present with growth failure and adults with diabetes insipidus and sexual dysfunction. Primary treatment consists of surgery. Following surgery, radiotherapy may be required.[bestpractice.bmj.com]
  • […] pediatric brain tumors (#3 after gliomas and medulloblastoma) Histoligically benign tumors Clinically present with headaches ( 50%), visual disturbances (40-70%), and pituitary dysfunction (up to 90%) Children: hypothyroid and GH deficiency Adults: sexual[en.wikibooks.org]
  • In comparison, sexual dysfunction is the most common endocrine problem in adults. Almost 90 percent of men complain of erectile dysfunction, while most women have amenorrhea.[aboutcancer.com]
  • Syndromes ranging from dwarfism in children to sexual dysfunction in adults may occur. Diabetes insipidus may occur in 25% of patients.[thedoctorsdoctor.com]

Workup

To evaluate craniopharyngioma diagnostically, precontrast and postcontrast CT (computed topography), MRA (magnetic resonance angiography), MRI (magnetic resonance imaging), neuro-ophthalmologic evaluation, neuropsychological assessment and complete endocrinology are used [9].

The MIB-1 labelling index

This index measures the proliferative activity of the condition. It is often determined by making use of an immunohistochemical method with monoclonal antibody MIB-1. This index comes in handy while planning adjuvant therapy. According to a study, a condition that shows an MIB-1 labelling index of higher than 7% shows has a high chance of regrowth/recurrence.

Enlarged Sella
  • Other signs include enlarged sella, bony erosion of the sella, dorsum, or clinoids, and signs of increased ICP. CT scan shows the extent of the lesion, distinguishes between solid, cystic and calcified components and demonstrates hydrocephalus.[thamburaj.com]
Slowing
  • Owing to the slow growth rate of these tumours, they are often quite large before becoming symptomatic. They are more common among children and older adults (55-74 years).[ncbi.nlm.nih.gov]
  • Craniopharyngioma (CP), a rare benign and slow-growing epithelial tumor, is mainly located within the sellar/parasellar region.[ncbi.nlm.nih.gov]
  • They are slow-growing tumours that can take 2-3 years (or longer) to manifest themselves before a diagnosis is made.[pituitary.org.uk]
  • Craniopharyngioma is a benign tumor and thought to be a slow growing tumor in childhood.[ncbi.nlm.nih.gov]
  • This tumor-type is one that exhibits slow growth. The symptoms often show insidiously and they do not become obvious until the tumor has attained a diameter of about 3cm.[symptoma.com]

Treatment

Treatment or non-treatment of this condition by a practitioner is dependent on the exact location, size, shape and the rate of growth. The need for removal becomes very urgent if the symptoms exhibited by the patient include visual loss.

Surgery is the primary method of treatment and this requires an open cranial procedure. Today, transnasal approaches that are minimally invasive have evolved and so the condition can be treated through the nose. Hormone replacement is used to treat any side effects of treatments and of the tumor itself.

Prognosis

Statistics show that the survival rates for craniopharyngioma is 86% at 2 years after detection and 80% at 5 years after diagnosis [10].

The rate of survival varies across age groups but the best rates have been reported for younger patients aged 20 and below. Older individuals aged 65% and above have a survival rate of 38%.

Etiology

A craniopharyngioma is a cystic tumor that is slow-growing, extra-axial, epithelial-squamous and calcified. It arises from the remains of the craniopharyngeal duct or the Rathke cleft (or both) and often occupies the suprasellar region [4].

The origin of craniopharyngioma is explained by two major hypotheses [5]. Both hypotheses explain the craniopharyngioma spectrum and both hypotheses are complementary.

The embryogenetic theory

This theory concerns how the adenohypophis develops and how the remnant ectoblastic cells of the craniopharyngeal ductis transformed. It also concerns the involuted Rathke pouch. The Rathke pouch and the infundibulum are developed during the fourth week of gestation and they both make up the hypophysis. By the second month, they elongate and come in contact.

The metaplastic theory

This theory is related to the residual squamous epithelium which undergoes metaplasia. The residual squamous epithelium is derived from the stomodeum and usually, part of the adenohypophysis.

The dual theory

This theory shows the spectrum of the craniopharyngioma. It attributes the adamantinomatous type of tumor which is mostly seen in children to embryonic remains while the squamous papillary or adult type is attributed to metaplastic foci. The metaplastic foci is arises from mature cells of the anterior hypophysis.

Epidemiology

In the United States, a study showed that the incidence for this condition is 0.18 per 100,000 individuals. No gender based variation has been found as well. The peak incidence is seen in adults aged 65-74 and children aged 5-14 [3].

Internationally, 1.4 cases per million are reported for children yearly. Generally, craniopharyngioma accounts for 1-3% of tumors in the cranium and it equally accounts for 13% of suprasellar tumors. In children, the condition represents 5-10% of all tumors and 56% of suprasellar and sellar tumors. Till date, there is no proof of definite genetic relationship there is very few records of familial cases.

The highest occurrences of intracranial tumors have been seen from Africa (18%), the Far East (16%) and Japan (10.5%).
In all age groups, a male predominance exists across all age groups (55%).

Generally craniopharyngiomas have a bimodal age distribution pattern. There is a peak between the ages 5 and 14 and in older adults aged 65 and above. However, the condition can be seen across individuals across all age groups.

Sex distribution
Age distribution

Pathophysiology

The condition arises from disordered embryogenesis partly as is the case with most sellar region tumors and Rathke’s cleft cysts.
However, the pathology of craniopharyngiomas and the Rathke’s cleft cysts differ [6]. This is what shows the varying ability of the squamous cells to either go through neoplastic change to form a craniopharyngioma or to remain as a normal cyst.

The pathology is complicated even further with the observation that particular craniopharyngiomas especially those seen in childhood often look like adamantinomas and odontogenic cysts.

Prevention

There are no guidelines for prevention of craniopharyngioma.

Summary

Craniopharyngioma is a brain tumor type which arises from the embryonic tissues of the pituitary gland. The condition is predominantly seen in children but also, it is seen with older adults aged 50-60 [1]. The specific cells from which the tumor originates are part and parcel of the tooth formation process so it is common to see calcium deposits on x-rays.

Other names usedin referring to Craniopharyngiomas include adamantinomas, hypophyseal duct tumors and the Rathke pouch tumors.

Craniopharyngiomas often start from the pituitary stalk and grows into the hypothalamus. The extension is done horizontally in line with the path that shows the least resistance. This growth is in various directions as shown below:

  • With anterior growth, it extends into the subfrontal spaces and the prechiasmatic cistern.
  • With posterior growth, it extends into the foramen magnum, the posterior fossa, third ventricle, cerebellopontine angle, interpeduncular cistern and the prepontine cistern.
  • With laterally growth it extends towards the subtemporal spaces

In some cases, the tumors extend towards the sylvian fissure. In very rare occasions, the tumors have been seen growing extracranially or extradurally leading to its development into a posterior fossa or nasopharyngeal craniopharyngiomas [2]. Another rare type of craniopharyngioma is the intraventricular craniopharyngioma.

Craniopharyngioma often looks like a collection of cysts or a single large cyst. The cysts are filled with a brownish yellow proteinaceous material which is glittery owing to the high amount of cholesterol crystals flowing in it.

The choice of approach to be used in tackling the tumor and its clinical behaviour is dictated by the primary location of the tumor and the extension pattern it has taken. Craniopharyngiomas that extend into the subfrontal spaces (prechiasmatic) and those that extend into the posterior fossa (retrochiasmatic) may not be diagnosed until they have grown in size.

Patient Information

A craniopharyngioma is a rare benign tumor that develops near the pituitary gland. This tumor most commonly occurs in children 5 - 10 years of age, but it can also occur in adults.

Common symptoms include:

References

Article

  1. Atlas SW. Magnetic Resonance Imaging of the Brain and Spine. Lippincott Williams & Wilkins. 2008 ISBN:078176985X.
  2. Saleem SN, Said AH, Lee DH. Lesions of the hypothalamus: MR imaging diagnostic features. Radiographics. 27 (4): 1087-108.
  3. Sartoretti-Schefer S, Wichmann W, Aguzzi A et-al. MR differentiation of adamantinous and squamous-papillary craniopharyngiomas. AJNR Am J Neuroradiol. 1997;18 (1): 77-87.
  4. Keating RF, Goodrich JT, Packer RJ. Tumors of the pediatric central nervous system. George Thieme Verlag. (2001) ISBN:0865778485
  5. Sekine S, Takata T, Shibata T, et al. Expression of enamel proteins and LEF1 in adamantinomatous craniopharyngioma:evidence for its odontogenic epithelial differentiation. Histopathology. Dec 2004;45(6):573-9.
  6. Sekine S, Shibata T, Kokubu A. Craniopharyngiomas of adamantinomatous type harbor beta-catenin gene mutations. Am J Pathol. Dec 2002;161(6):1997-2001.
  7. Garrè ML, Cama A. Craniopharyngioma: modern concepts in pathogenesis and treatment. Curr Opin Pediatr 2007; 19:471.
  8. Bunin GR, Surawicz TS, Witman PA, et al. The descriptive epidemiology of craniopharyngioma. J Neurosurg 1998; 89:547.
  9. Rickert CH, Paulus W. Lack of chromosomal imbalances in adamantinomatous and papillary craniopharyngiomas. J Neurol Neurosurg Psychiatry. Feb 2003;74(2):260-1.
  10. Rienstein S, Adams EF, Pilzer D, et al. Comparative genomic hybridization analysis of craniopharyngiomas. J Neurosurg. Jan 2003;98(1):162-4. 

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Last updated: 2018-06-22 07:12