Pheochromocytoma is a term used to refer to rare and mostly benign catecholamine-secreting tumors arising from the chromaffin cells found in the adrenal medulla or in the paraganglia (i.e. extra-adrenal pheochromocytoma). Clinical presentation can be highly variable and can mimic other diseases, but the condition typically presents with hypertension, palpitations, severe headaches and sweating.
As an entity, pheochromocytoma was initially described in 1886 by Frankel, following the autopsy of an 18-year-old that girl died suddenly in collapse, while the first successful surgical removal of the tumor was described in 1926 by Roux. Its name was established in 1912 by Pick and derived from Greek words “phaios” (meaning dusky), “chroma” (meaning
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Even though pheochromocytoma may occur in any age, the peak incidence is observed between the third and the fifth decade of life, with 10% of them occurring in children.
Etiology of pheochromocytoma
Pheochromocytoma usually occur as sporadic tumors, hence about 90% of them are presenting as unicentric and unilateral single adenomas. The remaining 10% of them are inherited, usually as bilateral and multiple, sometimes also found extra-adrenal (with similar histopathological traits).
Nevertheless, more than half of apparently sporadic pheochromocytomas found in patients with 18 years of age or younger are a result of an isolated identifiable mutation in germline DNA; this percentage can reach as high as 70% in children younger than 10. Thus guided genetic testing and genetic counseling is imperative for children presenting with pheochromocytoma, regardless of the family history.
Inherited pheochromocytomas may be isolated (inherited as an autosomal dominant trait) or a component of hereditary tumor syndromes – primarily multiple endocrine neoplasia (MEN) 2A and 2B and von Hippel-Lindau (VHL) disease, the familial paraganglioma syndromes and, in rare instances, MEN 1, neurofibromatosis (NF) type 1 and the tuberous sclerosis
Chordomas are cancerous tumors that occur anywhere on the spine. They grow slowly and can metastasize to other areas of the body. Other names for this disorder include CHDM, chordocarcinoma, chordoepithelioma, notochordal sarcoma, and notochordoma. Chordomas are rare and most often occur in people ages 40 to 70. Males are affected twice as often as females. About half of all chordoma cases appear at the base of the spine, while about a third occur at the base of the skull, and the rest appear in the kneck, upper back, or lower back vertebrae of the spine. Chordomas in the spine can cause the back, arms, or legs of the affected individual to feal weak or numb. Chordomas occurring at the base of the skull can cause double vision or headaches. Chordomas on the tailbone result in a large lump and can affect the function of the bladder or bowel..
Pituicytomas are rare, non-adenomatous tumors arising from the neurohypophysis that are classically comprised of banal spindle cells arranged in short fascicles or in a storiform pattern. More recently, pituicytomas have been found to express thyroid transcription factor-1 (TTF-1), a unifying feature of uncommon tumors arising from the posterior lobe of the pituitary gland that also include spindle cell oncocytoma and granular cell tumor of the sellar region. We describe a unique example of a twice recurrent pituicytoma received in consultation which exhibited an array of uncommon histomorphologic features. A recurrent heterogeneously enhancing 3 x 2 x 2 cm sellar tumor was transsphenoidally resected from a 68-year old male, status post two
People with this inherited disorder are likely to develop several kinds of tumors, including, in some cases, renal cell carcinoma.
Their analysis showed out of 240 cases 93.67% cases were non neoplastic 8.62% cases were neoplastic lesions and malignant lesions were
Follicular tumors, on the other hand, grow only on one side of the gland. These tumors develop in the hormone-producing cells and account for 30% - 50% of all thyroid cancers (Thackery 1057). Medullary thyroid tumors account for 5% - 7% of all thyroid cancers, and are usually uncommon. Like papillary tumors, they grow on one or both sides of the gland. Anaplastic tumors, though extremely rare, develop on either side of the thyroid gland and spread rapidly to other parts of the body. It account for only 2% of all thyroid cancers but is the fastest growing, and most aggressive thyroid cancer type (Thackery 1057).
Choroidal melanoma is the most common intraocular (inside the eye) tumor in adults. Choroidal melanoma is the most common cancer of the eye. It originates from the pigmented cells of the choroid of the eye, it is not like other tumors that starts in other parts of the body and then spreads to the eye.
Von Hippel Lindau (VHL) disease has characteristic tumors that result from germ mutations in the VHL tumor suppressor gene on chromosome 3p25.13 One of these tumors is the hemangioblastoma. These tumors consistently occur in “the retina, cerebellum, brainstem and dorsal spinal cord.”13 Hemangioblastomas are benign neoplasms with no potential to metastasize. They are space occupying lesions that can cause neurological deficit and can have a high mortality rate due to tonsillar herniation, compression of the brainstem, and hydrocephalus if left untreated. Hemangioblastomas may have a syrinx or pseudocyst evident with magnetic resonance imaging (MRI). Also on MRI, they can have the typical appearance of a “densely contrast-enhancing solid mass with smooth margins.”13 Central nervous
Pheochromocytoma (PCC) is a rare endocrine tumor involving the adrenal gland and is most common in preadolescent boys and adolescent girls. It is caused by pheochromocyte (chromaffin cells) neoplasms, which predominantly form in the adrenal medulla. However, they can also form in the extra-adrenal spaces, within the sympathetic paraganglia in the thoracic, abdominal and pelvic cavities. These neoplasms synthesize and secrete excess amounts of catecholamines, which produce symptoms - specifically hypertension, sweating, abdominal pain and weight loss (Ciftci et al., 2001; Estey and Diamandis, 2013). There have been improvements in the diagnosis and treatment of PCC in recent years. Usage of plasma and urine testing for catecholamines and their
Fig (4.6): 49-year-old woman with benign calcified breast mass. A, Spot compression magnification view of right breast shows small, ill-defined mass, containing clustered micro calcifications. B, Sonogram of mass shows an echogenic mass (arrows) with hypoechoic center. Biopsy yielded papillary apocrine metaplasia and calcifications associated with benign tissue. (Quoted from Soo MS.
Histopathology shows both solid and cystic components in varying proportions. The tumour has tubular lumina lined by cuboidal or columnar cells and variably sized cystic spaces. The solid portions contain two types of cells: polyhedral cells with basophilic cytoplasm and glycogen containing pale or clear cells with a clear cytoplasm and a round nucleus.The histology of malignant hidradenoma resembles that of its benign counterpart. The criteria for malignancy include poor circumscription, presence of nuclear atypia and mitotic activity, predominantly solid cell islands, infiltrative growth pattern, areas of necrosis and angio-lymphatic
These tumors arise from brain tissue called choroid plexus. They invade nearby tissue and spread via cerebrospinal fluid. There are two types of this tumor the noncancerous choroid plexus papilloma and the cancerous choroid plexus carcinoma. The symptoms of this cancer are headaches and increased pressure in the brain. These tumors represent two to four percent found in children under the age of fifteen. The cause of this cancer, like many others, is currently unknown. Surgery may be the only thing required for treatment. Completely removing the tumor relieves the hydrocephalus or the excess water in the brain about half the time. A drainage system may be required for others. Radiation and chemotherapy in choroid plexus tumors is still being defined. Radiation or chemo is recommended for patients with tumors not completely removed or not removeable. Second surgery might be recommended for recurrent tumors, followed with radiation or chemotherapy. The only known treatments that work for choroid plexus tumors are radiation and
Among the many types of cancer that can affect children, Neuroblastoma is very rare, yet it is the most common extracranial (located on the outside of the cranium) tumor in infants. It mostly affects children of five years and younger. Neuroblastoma is a kind of cancer that develops from neuroblasts (immature nerve cells). It usually appears around the adrenal glands in the kidneys, but might also be found in the chest, spine, abdomen, and neck.
Myoepithelioma is a rare benign salivary gland tumor which is also known as myoepithelial adenoma or benign myoepithelial tumor. It is only 1-1.5% of all tumors of the salivary gland (1). Credit for the identification of myoepitheloma as a distinct salivary gland tumor goes to Sheldon who was first identified this in 1943 (2). Myoepithelioma was recognized as a histologically distinct entity by the WHO in 1991 (3) .It is a benign salivary gland tumor entirely consist of Myoepithelial cells with different cellular morphologies including spindle, epithelioid, plasmacytoid or clear cells. Myoepithelioma can be composed of one or a mixture of those cell types.
Any lesion of the hypophysis or hypothalamus can result in secondary adrenal insufficiency; some of the examples are space-occupying lesions such as adenomas, craniopharyngiomas, sarcoidosis, fungal infections, trauma, and also metastases from distant malignant processes.
It is rarely found in children older than 10. This type of cancer accounts for about 7% of childhood cancers.