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Ectopic Parathyroid Adenoma in Piriform Sinus: Case Report and Review of the Literature.
Connolly, MJ, Lazinski, D, Aoki, KA, McLean, L, Torres, C, Dos Santos, MP
Ear, nose, & throat journal. 2019;(1):14-17
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Abstract
During routine blood work, a 53-year-old female patient was noted to have asymptomatic hypercalcemia and subsequently found to have hyperparathyroidism. Localization studies for a suspected parathyroid adenoma included 99mTc Sestamibi scintigraphy, Single Photon Emission Computed Tomography (SPECT)/computed tomography (CT) study, and ultrasound of the neck, which were initially read as negative for parathyroid adenoma. A contrast-enhanced CT scan of the neck was performed to locate the suspected parathyroid adenoma and demonstrated a soft tissue lesion within the right piriform sinus. Flexible fiber optic nasolaryngoscopy revealed a submucosal lesion in the right piriform sinus. Following these findings, the initial 99mTc Sestamibi scintigraphy and SPECT/CT were reviewed with confirmation of a focal area of increased activity superior to the right thyroid lobe, corresponding to a nodule in the right piriform sinus that demonstrated increased activity on SPECT/CT. The patient was brought to the operating room for surgical management where a laryngoscope and operating microscope were utilized. The encapsulated lesion was dissected and excised in total. The parathyroid hormone and ionized calcium levels normalized postoperatively. Pathology confirmed a parathyroid adenoma. Parathyroid adenomas are the most common cause of primary hyperparathyroidism. Sixteen percent of parathyroid adenomas can be situated in an ectopic location. Ectopic parathyroid adenomas in the piriform sinus are rare with only a few previously documented cases. We document a rare case of ectopic parathyroid adenoma in the piriform sinus overlooked on initial imaging studies. These lesions can be challenging to localize, however, an understanding of embryology, close scrutiny of possible ectopic locations, and the application of complementary imaging techniques may prove useful for surgeons and clinicians.
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2.
Cronkhite-Canada syndrome: a rare case report and literature review.
Zhao, R, Huang, M, Banafea, O, Zhao, J, Cheng, L, Zou, K, Zhu, L
BMC gastroenterology. 2016;:23
Abstract
BACKGROUND Cronkhite-Canada Syndrome (CCS) is a rare non-inherited disease characterized by gastrointestinal polyposis and ectodermal abnormalities, the estimated incidence is about one per million. Recognizing and curing the disorder face great challenge. CASE PRESENTATION This report refers to a Chinese 52 year old man with gastrointestinal symptoms and ectodermal abnormalities. Gastrointestinal symptoms occurred without obvious cause, followed by ectodermal abnormalities after two months. In several hospitals, endoscopy examinations found numerous polypoid lesions in various sizes spreading over the stomach and the entire colon and rectum, histopathological examinations showed inflammatory and adenomatous polyp. In our hospital, both endoscopy and the contrast-enhanced computed tomography (CT) of small intestine showed gastrointestinal polyposis. Gastric antrum and the colon biopsy samples suggested hyperplastic and inflammatory polyp respectively. Endoscopic ultrasonography (EUS) suggested gastric wall thickening. Fujinnon intelligent color enhancement (FICE) revealed that the size of gastric glands pit varied, and vessels were visible. Confocal endoscope showed increased glandular epithelium layers. Magnifying narrow-band imaging endoscopy (ME-NBI) detected that pit pattern in the mucous of the polyp were regular and type III-IV of microvessels were seen. Biochemical investigations showed anemia, hypoalbuminemia and electrolyte disturbance. IgG, IgA and C3 decreased. Anti-ribosomal phosphoprotein is weak positive. The patient was given nutritional support treatment. Gstrointestinal symptoms and hyperpigmentation improved gradually. CONCLUSION The patient was ever hospitalized in four hospitals and was diagnosed with CCS after 8 months of gastrointestinal symptoms. So when encountering the patient with gastrointestinal polyposis and ectodermal abnormalities, try to take CCS into consideration. Due to its low incidence, no standard therapy regimen has been established so far. However, nutritional support treatment is of great significance.
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Tumor suppressor gene mutation in a patient with a history of hyperparathyroidism-jaw tumor syndrome and healed generalized osteitis fibrosa cystica: a case report and genetic pathophysiology review.
Parfitt, J, Harris, M, Wright, JM, Kalamchi, S
Journal of oral and maxillofacial surgery : official journal of the American Association of Oral and Maxillofacial Surgeons. 2015;(1):194.e1-9
Abstract
Hyperparathyroidism-jaw tumor (HPT-JT) was first observed by Jackson in 1958 in a family who exhibited hyperparathyroidism and recurrent pancreatitis. The author noticed the presence of jaw tumors in the affected family and reported them as fibrous dysplasia. However, it was not until 1990 that a familial variety of hyperparathyroidism with fibro-osseous jaw tumors was recognized as HPT-JT syndrome and reported as a clinically and genetically distinct syndrome. Hyperparathyroidism generally arises from glandular hyperplasia or parathyroid adenomas, with only about 1% of cases resulting from parathyroid carcinoma. However, parathyroid carcinoma develops in about 15% of HPT-JT patients. The true incidence of HPT-JT is unknown, although the prevalence of about 100 published cases suggests its rarity. Twenty percent of HPT-JT cases have renal hamartomas or tumors, and female patients with HPT-JT have been reported to have carcinoma of the uterus. This syndrome appears to arise from a variety of mutations that deactivate the tumor suppressor gene CDC73 (also known as HRPT2) and its production of the tumor suppressor protein parafibromin. Functional parafibromin has 531 amino acids, and mutations result in a short nonfunctional protein. CDC73 disorders exhibit dominant germline gene behavior, with varying degrees of penetration. In most cases an affected person has 1 parent with the condition, which raises the need for family investigation and genetic counseling. We report a case of HPT-JT syndrome in a male patient who presented to the local community hospital 6 years previously with a history of back pain. Investigations showed elevated serum parathyroid hormone and calcium levels, and a technetium 99m sestamibi parathyroid scan showed increased activity at the site of the lower left gland that proved to be a substernal parathyroid carcinoma. The patient's parathyroid hormone level dropped from 126 to 97 pg/mL at 5 minutes and was 65 pg/mL at 10 minutes after excision of the gland, and the calcium chemistry findings returned to normal. Parathyroid histologic analysis showed substantial cytologic atypia with nuclear pleomorphism and prominent nucleoli, but infrequent mitoses. Although the capsule was described as showing foci of vascular invasion by the carcinoma, there has been no evidence of recurrence. Six years later, the patient presented with bilateral mandibular cemento-ossifying fibromas, but no evidence of hyperparathyroidism. The larger left tumor was excised and immediately reconstructed with an autogenous iliac crest bone graft, and the right lesion was enucleated. There has been no recurrence in 12 months. This case illustrates that the hyperparathyroidism and the fibro-osseous tumors are independent features of the persistent germline tumor suppressor gene (CDC73) mutation. The syndromic fibro-osseous tumors are odontogenic cemento-ossifying fibromas, which only occur in the jaws.
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Diagnostic and prognostic biomarkers of a sellar melanocytic tumor mimicking pituitary adenoma: Case report and literature review.
Mohammed, AA, Rotondo, F, Munoz, DG, Kovacs, K, Bilbao, JM, Karamchandani, JR, Di Ieva, A, Cusimano, MD
Pathology, research and practice. 2015;(9):682-7
Abstract
Primary or metastatic melanocytic tumors in the sellar region are rare and can pose a diagnostic challenge. Here we describe a case of a 74-year-old man who underwent radiological investigations for a transient episode of blurred vision. Based on the clinical and endocrinological findings and MRI results, the patient was assumed to have a clinically non-functioning pituitary macroadenoma, which was followed-up over a 2-year period. He did not have any endocrine symptoms or progressive visual deterioration, and no history of past malignancy, including melanoma. Endocrinological investigation was unremarkable; blood hormone levels were within the normal ranges except for low serum total testosterone and bioavailable testosterone levels without symptoms of hypogonadism. The longitudinal MRI follow-up demonstrated a gradual increase in the size of the tumor over the course of 11 months. For this reason, the patient underwent surgery. Pathologic examination including histology, immunohistochemistry and electron microscopy achieved the correct diagnosis of melanocytic tumor of the sellar region morphologic examination is essential in the diagnosis of melanocytic tumors. Hmb-45 is an important diagnostic biomarker in melanocytic lesions. The use and exploration of miRNA, Ki67 and osteopontin are important in understanding the genesis, progression, and prognosis in treatment of patients with melanocytic tumors.
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[Adrenal incidentaloma and nuclear medicine examination].
Tenenbaum, F
Journal de radiologie. 2009;(3 Pt 2):444-8
Abstract
In the setting of adrenal incidentaloma, nuclear medicine evaluation is only indicated after biological and imaging work-up has been completed. MIBG scintigraphy is helpful to characterize pheochromocytomas. In lesions without MIBG uptake, 18F FDG or 18F DOPA PET can be considered to characterize chromaffin cell tumours. To characterize lesions of the adrenal cortex, iodocholesterol scintigraphy is performed to confirm the origin of the adenoma and the benign or malignant nature of the lesion since benign adenomas show tracer uptake and malignant lesions show no tracer uptake. 18F FDG PET only characterizes the lesion as benign or malignant.
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An isolated pituitary metastasis as presentation of a differentiated hepatocellular carcinoma mimicking a nonfunctioning macroadenoma.
Moreno-Perez, O, Peiró, FM, López, P, Boix, E, Meoro, A, Serna-Candel, C, Aranda, FI, Picó, AM
Journal of endocrinological investigation. 2007;(5):428-33
Abstract
The differential diagnosis of sellar masses may be complex. Metastatic disease constitutes 1% of all pituitary lesions and sometimes mimics the clinical-radiological presentation of pituitary adenoma. The definitive diagnosis usually relies on histology, but occasionally even histological features of pituitary metastasis may resemble those of adenomas. We present a patient initially diagnosed with pituitary adenoma, but whose clinical course finally revealed pituitary metastasis of a hepatocellular carcinoma. The existing literature on this topic is reviewed.
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7.
Incidentalomas of the parathyroid gland: multiple presentations, variable function, and review of the literature.
Shroff, P, McGrath, GA, Pezzi, CM
Endocrine practice : official journal of the American College of Endocrinology and the American Association of Clinical Endocrinologists. 2005;(6):363-9
Abstract
OBJECTIVE To present a series of cases of parathyroid incidentalomas and review the related medical literature. METHODS The medical records of all patients of one surgeon who underwent unplanned removal of enlarged parathyroid glands found either during a surgical procedure performed for indications other than hyperparathyroidism or by ultrasonography of the thyroid gland between September 1989 and December 2003 were reviewed retrospectively. Clinical and laboratory data-- especially serum calcium and parathyroid hormone (PTH) levels--as well as information on postoperative parathyroid function are reported. RESULTS Among 421 patients (355 who underwent thyroidectomy and 66 who underwent transhiatal esophagectomy), 5 cases of parathyroid incidentalomas (1.2%) were identified. These incidental parathyroid adenomas were found during thyroidectomy for thyroid cancer in two patients, during esophagectomy for esophageal cancer in one patient (the first such reported case), and during ultrasonography of the neck for evaluation of thyroid nodules in two patients. Of the five patients, three had a normal and one had a slightly increased serum calcium concentration preoperatively; the serum calcium level was not determined preoperatively in the other patient. In one patient in whom intraoperative PTH level was determined both before and after resection of a large parathyroid adenoma, the initially high PTH value (180 pg/mL) declined to the normal range (48.2 pg/mL). In another patient, bone densitometry 9 months postoperatively showed an 8% increase in T-score for the lumbar spine and a 3.9% increase in T-score at the femoral neck, in comparison with bone density tests done 3 months preoperatively. No patient had persistent or recurrent hyperparathyroidism or hypoparathyroidism postoperatively or during subsequent follow-up. CONCLUSION Incidental identification of enlarged parathyroid glands during a neck surgical procedure is not common but does occur. Intraoperative and postoperative measurements of serum calcium and PTH along with postoperative bone density testing can help determine whether such parathyroid incidentalomas are functional. These enlarged parathyroid glands should be removed.
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8.
MEN-2A syndrome and pulmonary metastasis.
Alfaro, JJ, Lamas, C, Estrada, J, Lucas, T
Postgraduate medical journal. 2002;(915):51-2
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Abstract
A case of multiple endocrine neoplasia syndrome type-2A (MEN-2A) with primary hyperparathyroidism and medullary carcinoma of thyroid initially treated by surgery is reported. The presence of pulmonary nodules six years after the initial treatment was thought to be related to metastasis of medullary carcinoma, and the increase in serum calcium concentrations was assumed to be caused by persistence of parathyroid adenomatous or hyperplastic tissue. The patient underwent surgery again and the pulmonary nodules were confirmed to be metastases of a parathyroid carcinoma, a very rare entity in MEN-2A syndrome.
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9.
Pancreatitis secondary to hyperparathyroidism during pregnancy.
Dahan, M, Chang, RJ
Obstetrics and gynecology. 2001;(5 Pt 2):923-5
Abstract
BACKGROUND It has been suggested that magnesium can be used to reduce serum calcium levels seen with hyperparathyroidism during pregnancy, thus reducing maternal and fetal risk. CASE A young woman presented at 32 weeks' gestation with abdominal pain from pancreatitis caused by hyperparathyroidism from a parathyroid adenoma. She was started on magnesium sulfate tocolysis for preterm labor. During treatment, serum parathyroid hormone was undetectable, but serum calcium and vitamin D-1,25 were elevated. When magnesium was discontinued, her vitamin D-1,25 was suppressed and the parathyroid hormone was elevated. CONCLUSION For some patients, because of persistent hypercalcemia, magnesium sulfate might not be a viable treatment option for hyperparathyroidism during pregnancy.
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10.
Primary vaginal adenocarcinoma of intestinal type arising from an adenoma: case report and review of the literature.
Mudhar, HS, Smith, JH, Tidy, J
International journal of gynecological pathology : official journal of the International Society of Gynecological Pathologists. 2001;(2):204-9
Abstract
A 1 cm polypoid lesion was encountered on the posterior vaginal wall in a 56-year-old woman with no history of diethylstilbestrol exposure that on microscopic examination was a moderately differentiated adenocarcinoma of intestinal type. The tumor was cytokeratin 20 and carcinoembryonic antigen positive and negative for cytokeratin 7. Mucin histochemistry demonstrated the presence of o-acetylated sialomucin, a specific marker of large intestinal differentiation. The initial interpretation favored a metastasis from a colonic adenocarcinoma, but clinical investigations showed no evidence of a primary gastrointestinal lesion. The morphology, histochemical, and differential cytokeratin profile led to the lesion being reinterpreted as a primary intestinal-type adenocarcinoma of the vagina arising from a tubular adenoma. Although a very rare tumor, awareness of this lesion is important as it must be distinguished from metastatic adenocarcinomas from other sites.