Medullary Thyroid Carcinoma Manifesting as an Ovarian Mass: Case Report and Review of Literature

ObjectiveTo report the uncommon case of a woman with abdominal pain and a complex adnexal mass, who was subsequently found to have medullary thyroid carcinoma (MTC) metastatic to the ovary.MethodsWe present the clinical history, physical findings, laboratory and imaging studies, and pathologic findings in a woman with metastatic MTC and locally aggressive disease. The genetic associations, variable clinical course, and histopathologic findings in MTC are reviewed.ResultsA 38-year-old woman with abdominal and pelvic pain underwent a computed tomographic scan of the abdomen, which showed a complex left adnexal mass. After laparoscopic left oophorectomy, histopathologic analysis of the resected ovary suggested the presence of a metastatic neuroendocrine tumor. The patient recovered but was referred to the surgery clinic 3 months later because of hoarseness, a left neck mass, and left-sided vocal cord paralysis. Further work-up was suggestive of MTC, which prompted RET testing for multiple endocrine neoplasia. The patient underwent left thyroid lobectomy and selective lymph node dissection but later required tracheostomy because of tumoral invasion of the trachea, laser debulking of the tumor, and external beam radiation therapy. One year postoperatively, development of a metastatic lesion in her right ovary necessitated a second oophorectomy.ConclusionMTC usually manifests as a solitary thyroid nodule but should be considered in patients with metastatic lesions characterized by neuroendocrine features. This unusual case highlights the biologic and clinical variability of this often aggressive thyroid cancer, which necessitates an attentive work-up, a rigorous operative strategy, and a periodic postoperative surveillance program. (Endocr Pract. 2008;14:351-357)     

Orbital Inflammatory Disease in A Patient Treated with Zoledronate

ObjectiveTo increase awareness of orbital inflammatory disease as a rare adverse effect of bisphosphonates.MethodsWe present a case report and a review of the relevant literature.ResultsA 57-year-old woman with history of esophageal, breast, and lung cancers was diagnosed with postmenopausal osteoporosis. She initially received intravenous ibandronate for a total of 6 infusions. Later, she was changed to zoledronate infusion because of its yearly dosing schedule. Several hours after her initial infusion of zoledronate, she developed a painfully swollen left eye with photophobia. Ophthalmologic exam showed edema of the left upper lid. No exophthalmos was noted. Slitlamp exam showed conjunctival injection in the left eye with an elevated intraocular pressure. An orbital computed tomographic scan showed inflammation of the left orbital, preseptal, and retroseptal spaces. She was started on 2 methylprednisolone dose packs and the swelling and erythema disappeared completely in 2 weeks. Subsequent orbital magnetic resonance imaging showed no mass within either the left or right orbit, and no abnormal enhancement following contrast administration.ConclusionPhysicians should be aware of this rare complication of zoledronate. It should be used with caution in patients with a history of inflammatory eye disease or mild ocular symptoms following use of a bisphosphonate. (Endocr Pract. 2011;17:e101-e103)     

Treatment of Osteoporosis and Prevention of New Fractures: Role of Intravenously Administered Bisphosphonates

ObjectiveTo evaluate the usefulness of intravenously administered bisphosphonates for improving absorption, tolerability, adherence, and outcomes in the treatment and prevention of osteoporosis.MethodsData published from 1996 to 2009 relevant to the treatment of osteoporosis, with emphasis on bisphosphonates, fracture risk, adherence to therapy, frequency of dosing, intravenous treatment, tolerability, cost-effectiveness, and quality of life, were reviewed.ResultsAlthough bisphosphonates are currently the standard of care for treatment of postmenopausal osteoporosis and osteoporosis in men, oral formulations are associated with poor absorption and potential irritation of the upper gastrointestinal tract. These issues necessitate complicated and restrictive dosing regimens, which in turn lead to poor compliance and persistence. Intravenous formulations such as 3 mg of ibandronate given quarterly and 5 mg of zoledronic acid administered once yearly avoid problems relating to absorption and tolerability by bypassing the gastrointestinal tract. Intravenously administered ibandronate is presumed (by virtue of similar or superior improvements in bone mineral density) to have antifracture efficacy similar to that of orally administered ibandronate given daily, which has been shown to produce significant reductions in vertebral fractures during a 3-year period in comparison with placebo. Zoledronic acid, 5 mg once yearly, has been shown to produce a significant reduction in the risk of morphometric vertebral fractures, clinical vertebral fractures, hip fractures, and nonvertebral fractures versus placebo during a 3-year interval in patients with postmenopausal osteoporosis and also to yield a significantly decreased risk for new clinical fractures versus placebo in patients with recent low-trauma hip fracture. Both agents have favorable safety and tolerability profiles.ConclusionIntravenously administered bisphosphonates have the potential to increase compliance and persistence with therapy in patients with osteoporosis and to improve patient outcomes. (Endocr Pract. 2009;15:483-493)     

Oncogenic Osteomalacia Cured by Removal of an Organized Hematoma

ObjectiveTo describe a patient with oncogenic osteomalacia whose symptoms were rapidly resolved after surgical removal of an organized hematoma of the hip.MethodsA case report is presented, including clinical and laboratory findings. The relevant literature is reviewed, and the current understanding of oncogenic osteomalacia is summarized.ResultsIn September 1996, a 44-year-old black woman presented with a 2-year history of bone pain, progressive muscle weakness, depression, osteomalacia, and hypophosphatemia. Her condition did not improve with use of calcitriol and phosphate replacement. During the previous year, her serum phosphorus levels were low, ranging from 1.0 to 2.2 mg/dL, and the levels of serum 1,25-dihydroxyvitamin D [1,25-(OH)₂D] were very low, ranging from < 5 to 19.4 pg/mL (normal, 15 to 60). The serum 25-hydroxyvitamin D levels were low, ranging from 8 to 14 ng/mL (normal, 9 to 52). The higher values were noted after she had received large doses of phosphate, 1,25-(OH)₂D, and vitamin D. During the previous year, her serum alkaline phosphatase levels were high, ranging from 253 to 314 U/L; serum calcium and parathyroid hormone levels were normal. The abnormalities on physical examination were obesity and a 10- by 10-cm firm, poorly demarcated mass superior to the left greater trochanter. A computed tomographic scan of this region showed a water-density fluid collection in the left buttock measuring 7.8 by 7.8 cm, consistent with a chronic hematoma. The mass was resected, and histopathologic examination revealed features of an organized hematoma with areas of myxoid changes and cartilaginous metaplasia. Postoperatively, the patient’s strength improved, and the levels of serum phosphorus and 1,25-(OH)₂D became supranormal.ConclusionThe symptoms and laboratory abnormalities of this patient with oncogenic osteomalacia promptly resolved after resection of an organized hematoma of the left hip. (Endocr Pract. 2005;11:190-193)     

Idiopathic Hypoparathyroidism Presenting with Severe Hypocalcemia and Asymptomatic Basal Ganglia Calcification Followed by acute Intracerebral Bleed

ObjectiveTo report a case of idiopathic hypoparathyroidism presenting with severe hypocalcemia and intracerebral calcifications that resulted in a spontaneous intracerebral bleed.MethodsWe present the clinical, laboratory, and radiologic findings in a woman with idiopathic hypoparathyroidism who developed spontaneous intracerebral bleed in the setting of chronic intracerebral calcifications.ResultsA 37-year-old woman presented with vague symptoms of hypocalcemia. Clinical evaluation revealed brisk deep tendon Reflexes and positive Chvostek’s and Trousseau’s signs. The serum calcium level was 3.7 mg/ dL (reference range, 8.0 to 10.6 mg/dL) and the phosphorus level was 8.2 mg/dL (reference range, 2.3 to 5.0 mg/dL). Serum intact parathyroid hormone was undetectable. Computed tomography of the head showed extensive bilateral symmetrical calcification of basal ganglia and dentate nucleus in the cerebellum and centrum semiovale. Fluid and electrolytes were replaced appropriately, and calcium and calcitriol were prescribed. While in the hospital, the patient developed an acute intracerebral bleed confirmed by computed tomography. The patient recovered without neurologic sequelae and was discharged from the hospital on calcium supplementation and calcitriol. Repeated computed tomography of the head 3 years later demonstrated complete resolution of the bleed.ConclusionThis case suggests that patients with severe hypoparathyroidism and intracerebral calcification may be at risk for spontaneous intracerebral bleed and should be monitored accordingly. (Endocr Pract. 2007; 13:487-492     

Recurrent Acute Suppurative Thyroiditis Attributable to a Piriform Sinus Fistula in an Adult

ObjectiveTo report a case of recurrent acute suppurative thyroiditis (AST) caused by a piriform sinus fistula (PSF).MethodsWe review the related literature and describe the clinical presentation, diagnostic evaluation, and management of a patient with recurrent AST due to PSF.ResultsA 50-year-old woman presented with recurrent episodes of pain and swelling in the left anterior neck area and odynophagia. The initial episode, 1 year previously, lasted for 1 month, and her condition improved with a short course of prednisone. Subsequently, the neck pain recurred and extended upward to both sides of the neck. She was suspected of having thyroiditis and was treated with prednisone for 6 weeks, but the symptoms persisted. Physical examination showed tenderness and induration of the skin over the left lobe of the thyroid. Ultrasonography disclosed an ill-defined solid-cystic area in the left thyroid lobe. Fine-needle aspiration cytology revealed acute inflammation, consistent with an infected cyst. A computed tomographic scan of the neck showed obscuration of the fat planes involving the left strap musculature and evidence suggestive of microabscesses. Prolonged intravenous antibiotic treatment ultimately alleviated the symptoms. Direct laryngoscopy revealed a fistula extending anteriorly from the apex of the piriform sinus. The patient underwent complete fistulectomy and left thyroid lobectomy and was asymptomatic on followup.ConclusionPSF should be suspected as a cause of recurrent AST. Absence of fever, toxemia, and local erythema does not rule out the condition. Unresponsiveness to corticosteroid therapy in thyroiditis should suggest the diagnosis. Complete fistulectomy and resection of the involved thyroid lobe result in permanent cure. (Endocr Pract. 2007;13:662-666)     

Cushing Disease Revealed by Bilateral Atypical Central Serous Chorioretinopathy: Case Report

ObjectiveWe report the case of a patient with Cushing disease revealed by bilateral central serous chorioretinopathy (CSCR).MethodsWe present the clinical history, physical findings, laboratory results, and imaging studies of a 53-year- old Chinese woman with a Cushing disease revealed by bilateral CSCR. The association with CSCR and the pertinent literature are reviewed.MethodsWe present the clinical history, physical findings, laboratory results, and imaging studies of a 53-year- old Chinese woman with a Cushing disease revealed by bilateral CSCR. The association with CSCR and the pertinent literature are reviewed.ResultsA 53-year-old patient initially presented to the Department of Ophthalmology with a 4-week history of decreased vision in the left eye. Standard ophthalmologic examination and fluorescein angiography established the diagnosis of bilateral CSCR. Systemic clinical signs and biochemical analysis indicated hypercortisolism. Magnetic resonance imaging (MRI) of the pituitary gland showed a left-side lesion compatible with a microadenoma. The diagnosis of Adrenocorticotropic hormone (ACTH)-dependent Cushing syndrome secondary to a pituitary microadenoma was selected. Endoscopic endonasal transsphenoidal surgery was performed and the pituitary adenoma was successfully removed. The histology confirmed the presence of ACTH-immunopositive pituitary adenoma. Early postoperative morning cortisol levels indicated early remission. At 6 weeks postoperatively, the patient’s morning cortisol remains undetectable, and serous retinal detachments had regressed.ConclusionCSCR is an uncommon manifestation of endogenous Cushing syndrome. It can be the first presentation of hypercortisolism caused by Cushing disease. CSCR should be considered when assessing patients with Cushing syndrome complaining of visual disorders. On the other hand, it is useful in patients with an atypical form of CSCR to exclude Cushing’s syndrome. (Endoer. Praet. 2013;19:el29-el33)     

Unusual Case of Metastatic Neuroendocrine Tumor

ObjectiveTo report a rare case of metastatic growth hormone (GH)-secreting pituitary carcinoma causing acromegaly.MethodsWe present a case report and review the available literature on this topic.ResultsA 68-year-old woman presented with persistent acromegaly after treatment for a GH-secreting pituitary adenoma. Evaluation of long-standing cervical adenopathy revealed findings consistent with a metastatic neuroendocrine tumor. Further work-up revealed additional thyroid, parathyroid, and cervical masses. After operative treatment including total thyroidectomy, subtotal parathyroidectomy, partial thymectomy, and right modified radical neck dissection, the patient’s symptoms diminished, and her GH levels approached the normal range. Surgical pathology findings were consistent with a GH-secreting pituitary carcinoma metastatic to the cervical lymph nodes, multinodular thyroid hyperplasia with a focus of papillary microcarcinoma, and parathyroid hyperplasia.ConclusionOverall, pituitary carcinomas are extremely rare. To date, about 100 cases have been reported in the world’s literature, and of these, only 19 cases originated from GH-secreting cells. Our examination of the symptoms, signs, diagnosis, and treatment of our patient, in comparison with the previously reported cases, should enhance awareness of this unusual disease process. (Endocr Pract. 2007;13:72-76)     

A Case of Riedel’s Thyroiditis Treated with Tamoxifen: Another Successful Outcome

ObjectiveTo report a case of Riedel’s thyroiditis, which was successfully treated with tamoxifen.MethodsWe present the clinical, laboratory, and imaging findings and describe the clinical course of a patient with Riedel’s thyroiditis.ResultsA 40-year-old woman presented with hypothyroidism and a large goiter, which was unresponsive to hormone replacement therapy. Magnetic resonance imaging confirmed the presence of an enlarged thyroid gland, more pronounced on the right than on the left. The patient had progressive discomfort attributable to compressive symptoms in the neck. Surgical exploration of the neck disclosed a hard, immobile thyroid mass, which could not be resected because of adherence to surrounding structures. Biopsy of the thyroid and of the muscles of the neck revealed Riedel’s thyroiditis. Treatment with tamoxifen, in a dosage of 20 mg twice a day for more than 1½ years, completely resolved the neck mass (substantiated by follow-up magnetic resonance imaging) and relieved the signs and symptoms of compression of the neck.ConclusionTamoxifen treatment is effective in resolving the mass and compression in Riedel’s thyroiditis. (Endocr Pract. 2004;10:483-486)     

Hyperthyroidism due to Coexistence of Graves’ Disease and Struma Ovarii

ObjectiveTo report an unusual case of persistent thyrotoxicosis after treatment of Graves’ disease, because of coexistence of struma ovarii.MethodsWe report the clinical history, imaging studies, laboratory and pathologic data, and treatment in a patient with persistent hyperthyroidism after surgical treatment of Graves’ disease. In addition, we discuss some aspects of the pathogenesis of hyperthyroidism due to functioning struma ovarii.ResultsA 42-year-old woman underwent near-total thyroidectomy for treatment of Graves’ disease. Post-operatively, hyperthyroidism was still present. Methimazole was administered again, and performance of a ¹³¹I whole-body scan demonstrated a focus of intense uptake in the pelvis. Pelvic ultrasonography revealed a mass (11 by 8 by 7.1 cm) arising from the right ovary, with both solid and cystic components. Abdominal surgical exploration was performed, and the final histologic diagnosis was struma ovarii. The symptoms of hyperthyroidism diminished, and 3 weeks postoperatively, the thyroid hormone levels were in the hypothyroid range.ConclusionIn patients with refractory hyperthyroidism after thyroid surgical treatment, radioiodine scanning should be performed to diagnose or exclude the functioning profile of ovarian masses. (Endocr Pract. 2007;13:274-276)     

Three large, functioning cystic parathyroid adenomas

ObjectiveTo report a very rare case of 3 large, functioning cystic parathyroid adenomas causing primary hyperparathyroidism.MethodsWe present the history, clinical findings, laboratory test results, radiologic findings, endocrine workup results, intraoperative surgical challenges, and surgical pathology report of the study patient. We review the literature and discuss the importance of intraoperative parathyroid hormone (PTH) measurement in such cases.ResultsA 79-year-old woman presented with primary hyperparathyroidism and elevated levels of calcium and PTH. Localization studies confirmed the presence of a large right upper parathyroid adenoma. On exploration, a very large cystic parathyroid gland was identified at that location. Because intraoperative PTH levels remained elevated, further exploration was pursued, which revealed 2 more large cystic glands on the left side that were resected. This resulted in an adequate but slow PTH drop. The right lower gland appeared normal. On follow-up 4 days and 6 weeks after surgery, the calcium and PTH levels had normalized.ConclusionThis case highlights the aspects of intraoperative PTH use and underscores the need to exclude multigland disease even in the setting of a very large parathyroid cyst with concordant localization studies. (Endocr Pract. 2012;18:e14-e16)     

Bisphosphonate Treatment in Polyostotic Fibrous Dysplasia of the Cranium: Case Report and Literature Review

ObjectiveTo report the case of a patient with polyostotic fibrous dysplasia of the cranium who showed dramatic improvement after treatment with intravenous zoledronic acid.MethodsWe present the clinical findings, laboratory test results, surgical pathology report, and imaging studies of a man with extensive fibrous dysplasia of the cranium and review the literature regarding the use of bisphosphonates in patients with this debilitating skeletal disorder.ResultsA 32-year-old man presented with chronic occipital headache, and computed tomography of the head revealed extensive bony lesions of the middle and posterior cranial fossa. Bone biopsy confirmed the diagnosis of fibrous dysplasia. Laboratory blood test results revealed elevated serum alkaline phosphatase (140 U/L [reference range, 39-117 U/L]) and elevated serum bone-specific alkaline phosphatase (30.6 μg/L [reference range, 6-20 μg/L]). The patient was treated with intravenous pamidronate without improvement, and therapy was switched to zoledronic acid, which resulted in rapid resolution of headache symptoms, decrease in bone-specific alkaline phosphatase levels to 24.9 μg/L, and dramatic radiologic improvement on repeated computed tomography of the head.ConclusionsThe treatment options for fibrous dysplasia of the cranium have been limited to conservative follow-up or surgery, and the use of zoledronic acid in this condition has not been previously reported. Intravenous bisphosphonate therapy is suggested to be a useful option in the treatment of cranial fibrous dysplasia. (Endocr Pract. 2010;16:851-854)     

Hyperandrogenism Due to a Testosterone-Secreting Sertoli-Leydig Cell Tumor Associated With a Dehydroepiandrosterone Sulfate-Secreting Adrenal Adenoma in a Postmenopausal Woman: Case Presentation and Review of Literature

ObjectiveTo report a case of hyperandrogenism attributable to the presence of an adrenal adenoma secreting dehydroepiandrosterone sulfate (DHEA-S) and an ovarian Sertoli-Leydig cell tumor secreting testosterone in a postmenopausal woman.MethodsThe laboratory, radiologic, and pathologic findings in our case are described. In addition, the pertinent literature is reviewed.ResultsA 56-year-old woman presented with a history of gradual increase in facial and body hair, scalp hair loss, male pattern baldness, and deepening of her voice, beginning a few years after spontaneous menopause at age 49 years. She had hypertension, obesity, and type 2 diabetes mellitus. Laboratory tests showed elevated levels of total testosterone (348 ng/dL) and DHEA-S (2,058 μg/dL), and a left adrenal tumor (3 by 4 cm) was detected on abdominal computed tomographic scan. Laparoscopic left adrenalectomy was performed, and the pathologic diagnosis was adrenal adenoma. The DHEA-S returned to normal levels, but the serum testosterone concentration remained elevated. Transvaginal ultrasonography disclosed an ovarian tumor. Bilateral oophorectomy was performed, and an ovarian Sertoli-Leydig cell tumor was diagnosed. The hormonal and clinical picture normalized after this surgical intervention.ConclusionAfter extensive review of the literature, we believe that this is the first reported case of a coincidental DHEA-S-secreting adrenal adenoma and a testosterone-secreting ovarian Leydig cell tumor causing signs of virilization. (Endocr Pract. 2009;15:149-152)     

Thyrotoxicosis due to Ectopic Lateral Thyroid Tissue Presenting 5 Years After Total Thyroidectomy

ObjectiveTo describe a patient who, 5 years after total thyroidectomy to treat Graves disease, presented with thyrotoxicosis due to nonmalignant lateral ectopic thyroid tissue.MethodWe describe the laboratory, imaging, and physical findings of the study patient and review the relevant literature.ResultsA 32-year-old white woman with a history of Graves disease presented with recurrent hyperthyroidism 5 years after total thyroidectomy. A radioactive iodine scan was performed, which revealed elevated uptake (40%) and positive imaging in the left mid-neck. Ultrasonography examination of the neck confirmed the absence of any thyroid tissue within the thyroid bed, but documented 2 nodular, hypoechoic left upper-neck masses with punctuate hyperlucency. Contrast-enhanced computed tomography was performed to precisely localize the nodules, which were excised surgically via selective neck dissection. Histopathologic examination revealed chronic lymphocytic inflammatory infiltrate with focal thyroid hyperplasia and papillary infoldings and no evidence of malignancy.ConclusionsTo our knowledge, this represents the first report of ectopic benign thyroid tissue as the sole cause of hyperthyroid symptoms, and this entity should be considered in patients who have undergone thyroidectomy and have persistent hyperthyroidism. (Endocr Pract. 2011;17:70-73)     

Transformation of a Pituitary Macroadenoma into to a Corticotropin-Secreting Carcinoma Over 16 Years

ObjectiveTo describe a case of a pituitary macroadenoma that differentiated into a corticotropin (ACTH)-secreting carcinoma with metastasis to the thigh.MethodsWe present a case report with a 16-year follow-up that includes anatomic and endocrine documentation of the history of an ACTH-secreting carcinoma.ResultsA 32-year-old woman presented for evaluation in 1989 because of visual feld defects. Magnetic resonance imaging revealed a locally invasive 3-cm macroadenoma. She had no clinical signs of cortisol excess. The patient underwent surgical debulking followed by a course of radiation directed to the pituitary. Results from retrospective immunohistochemical staining with antibodies against ACTH, prolactin, and MIB-1 were negative. Postoperatively, she could not be weaned from exogenous steroids without developing symptoms of adrenal insuffciency. In 1995, she developed left facial palsy and diplopia caused by tumor growth. In 1997, the patient developed progressive symptoms of cortisol excess, which continued after exogenous steroid replacement was discontinued. The patient’s clinical status continued to deteriorate because of local mass effect from tumor growth and uncontrolled hypercortisolism. She underwent bilateral adrenalectomy in 2003. The patient remained debilitated in a long-term care facility for 2 years when she was found to have a mass on her left hip. Biopsy results of the obturator muscle revealed metastatic tumor of neuroendocrine origin with strong reactivity to ACTH antibodies and MIB-1 labeling in 8% of tumor cell nuclei.ConclusionA pituitary tumor can transform into an ACTH-secreting carcinoma in an indolent manner. Patients with invasive pituitary adenomas require long-term surveillance to monitor for differentiation into pituitary carcinoma. (Endocr Pract. 2007;13:463-471)     

Transient Thyrotoxicosis Following Minimally Invasive Radio-Guided Parathyroidectomy

ObjectiveWe report a case of thyrotoxicosis following minimally invasive parathyroidectomy.MethodsCase report with literature review.ResultsA 70-year-old woman with primary hyperparathyroidism developed thyrotoxicosis 2 weeks following minimally invasive radio-guided parathyroidectomy. A diagnosis of postparathyroidectomy transient thyrotoxicosis (PTT) was made after excluding other causes of thyrotoxicosis. The patient reverted back to a euthyroid state in 4 weeks with conservative management.ConclusionPTT should be considered in the differential diagnosis in patients developing features of thyrotoxicosis following parathyroidectomy. (Endocr Pract. 2014;20:e1-e3)     

Cushing Syndrome in a Young Woman Due to Primary Pigmented Nodular Adrenal Disease

ObjectiveTo report a case of Cushing syndrome due to apparently sporadic primary pigmented nodular adrenal disease in a young woman.MethodsWe describe the clinical, biochemical, radiologic, and histologic findings of Cushing syndrome due to the rare condition of primary pigmented nodular adrenal disease.ResultsA 30-year-old woman presented with a 2-year history of worsening itch without rash over her shoulders and arms and weight gain, particularly around the abdomen and face. Careful questioning did not elicit any history of exogenous glucocorticoid use (systemic or topical), including hydrocortisone. On examination, the patient had a slightly rounded and plethoric face, a small buffalo hump, central adiposity, and thin skin with a few small striae on her inner thighs. No features of the Carney complex were observed. Investigations showed hypercor- tisolism with suppressed corticotropin and normal adrenal imaging despite documentation of enlarged adrenal glands at removal. High-dose dexamethasone administration was followed by a decrease in urinary free cortisol excretion rather than a paradoxical rise as previously reported in primary pigmented nodular adrenal disease. No mutations were detected in the PRKAR1A gene.ConclusionsPrimary pigmented nodular adrenal disease should be suspected in patients with corticotropinindependent Cushing syndrome who have normal adrenal imaging. The role of genetic testing in apparently sporadic cases is not established, but cumulative experience may be helpful in defining the frequency of PRKAR1A mutations. (Endocr Pract. 2010;16:84-88)     

Pulmonary Atypical Carcinoid Tumor with Metastatic Involvement of the Pituitary Gland Causing Functional Hypopituitarism

ObjectiveTo report a unique case of a peripherally located pulmonary atypical carcinoid tumor with metastatic involvement of the pituitary, manifesting with evidence of functional hypopituitarism and compressive symptoms of dysarthria, bitemporal loss of vision, and ataxia.MethodsWe present a case report, including detailed laboratory, radiologic, and pathologic findings in a 50-year-old woman with a peripherally located pulmonary atypical carcinoid tumor and a lesion metastatic to the pituitary gland. The pertinent literature is also reviewed.ResultsA 50-year-old woman with a medical history of metastatic pulmonary atypical carcinoid tumor presented with symptoms of bitemporal hemianopia, ataxia, and dysarthria. Laboratory evaluation revealed functional hypopituitarism, and magnetic resonance imaging of the brain with use of gadolinium contrast demonstrated a pituitary lesion measuring 3.5 by 2.5 by 2.5 cm. Visual field testing revealed bitemporal superior quadrantanopia, consistent with a pituitary lesion of the chiasm. Computed tomographic angiography excluded the presence of an aneurysm of the internal carotid artery. Subsequently, the patient underwent subtotal transsphenoidal hypophysectomy. Findings on pathology examination were consistent with a lesion metastatic from the primary pulmonary atypical carcinoid tumor. The patient subsequently received monthly octreotide injections as well as chemotherapeutic regimens of capecitobine and temozolomide. At 18-month follow-up, disease had not recurred.ConclusionThis case of metastatic involvement of the pituitary gland from a peripherally located pulmonary atypical carcinoid tumor manifesting with evidence of functional hypopituitarism is highly uncommon. To our knowledge, such a case has not been previously reported. The presentation of an advanced pituitary metastatic lesion can be particularly dramatic, and the course can be potentially complicated. Therefore, heightened awareness of the possibility of a pituitary lesion metastatic from a pulmonary atypical carcinoid tumor is important. (Endocr Pract. 2007;13:291-295)     

Nonfunctioning Parathyroid Carcinoma: Case Report and Review of Literature

ObjectiveTo report a case of nonfunctioning parathyroid carcinoma that was incidentally found during a thyroidectomy for multinodular goiter.MethodsWe present a case report, detailing the clinical course and histologic findings in a patient with a nonfunctional parathyroid carcinoma. The related literature is also reviewed.ResultsA 67-year-old woman presented with a 30-year history of a multinodular goiter that was symptomatic. A total thyroidectomy was performed. Histologic examination revealed not only a multinodular thyroid but also a mass in the left lobe, which was diagnostic of a parathyroid carcinoma. Serum calcium and parathyroid hormone levels were normal postoperatively. Eleven months after the initial operation, a suprasternal mass developed, and she underwent neck reexploration and subtotal resection of an invasive recurrent nonfunctioning parathyroid carcinoma. The serum parathyroid hormone and calcium levels were normal before and after the operation. Postoperatively, the patient underwent radiation therapy. Twenty-three months after the initial operation, a computed tomographic scan of the chest revealed an interval increase in size of a nodule in the left lower lobe of the lung, and 30 months after her initial operation, she underwent resection of an isolated, 1-cm (greatest diameter), metastatic parathyroid carcinoma in the left lower lobe of the lung. The patient is currently doing well without evidence of recurrent disease.ConclusionNonfunctioning parathyroid carcinomas are difficult to diagnose and to treat. Recurrent disease after operation is common, and radiation therapy may help stabilize tumor growth. Patients with nonfunctioning parathyroid carcinomas appear to have a poorer prognosis than do those with functioning parathyroid cancers. (Endocr Pract. 2007;13:750-757)     

Hypertensive Crisis,Catecholamine Cardiomyopathy,and Death Associated with Pseudoephedrine use in a Patient with Pheochromocytoma

ObjectiveTo describe a woman with a previously unrecognized pheochromocytoma who died after ingesting over-the-counter pseudoephedrine-containing medications.MethodsWe present a case report including laboratory, radiographic, and pathologic findings in a patient with a previously unrecognized pheochromocytoma.ResultsA 31-year-old woman had symptoms consistent with intermittent, excessive release of catecholamines since childhood. She developed an upper-respiratory infection and used over-the-counter medications containing pseudoephedrine. Subsequently, she developed a hypertensive crisis with congestive heart failure and died of a cardiac arrhythmia and shock. Findings from postmortem examination included a right adrenal pheochromocytoma, congestive heart failure, and catecholamine cardiomyopathy.ConclusionThis patient death may be linked to the use of pseudoephedrine hydrochloride. Physicians and pharmacists should warn patients with known pheochromocytoma or those at risk for having pheochromocytoma on the basis of family history or genetic testing that pseudoephedrine use may be harmful. Over-the-counter packaging should include such warning. (Endocr Pract. 2008; 14:93-96)