Thymus Carcinoid

Viebahn, R.; Hiddemann, W.; Klink, F.; Bassewitz, D. B. v.

doi:10.1016/s0344-0338(85)80119-9

Cited by 26 publications

(4 citation statements)

References 9 publications

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“…1,2 In the thymus, neuroendocrine carcinomas account for approximately 2% to 4% of all anterior mediastinal tumors. 3,4 Neuroendocrine carcinomas of the thymus associated with Cushing's syndrome can occur at any age between 4 and 64 years, but their peak prevalence is between the second and the fourth decades of a patient's life, 5 as frequently in men as in women. 6,7 Diagnosis must be confirmed by increased cortisol levels in the serum or in the 24-hour urine collection, which must remain elevated despite a low-dose dexamethasone suppression test.…”

Section: Discussionmentioning

confidence: 99%

Thymic Carcinoid Presenting With Cushing's Syndrome in a 17-Year-Old Boy: A Case Report and Review of the Literature

McEvoy

Rich

New

et al. 2011

JCO

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A 17-year-old boy was referred to our institution for evaluation of Cushing's syndrome. At presentation, he had experienced muscle weakness, a 10-to 15-pound weight gain within a few months, and facial puffiness. He had also developed acne and increased body hair, and he was only able to sleep 4 to 5 hours a night. He felt fatigued and depressed. Although he deliberately lost 15 pounds in the course of 3 months, his face remained full. He underwent laboratory tests, and his test results were negative for Lyme disease, mononucleosis, and thyroid deficiency. He was finally diagnosed with Cushing's syndrome.On physical examination, his blood pressure was 138/70 mmHg, and he had a pulse rate of 85 beats per minute. His height was in the 48th percentile. His weight was in the 25th percentile. He had a cushingoid appearance: acne and hyperpigmented macules on the back and violaceous striae on the elbows, knees, and hips. He also had a hyperpigmented and thinning transverse scar on his back, thinning scalp hair, and bilateral pedal edema. He was in Tanner stage V of development.Initial laboratory testing revealed a serum cortisol level of 24.4 mcg/dL (normal range, 3.1 to 16.7 mcg/dL), a morning serum cortisol level of 30.5 mcg/dL (normal range, 6 to 23 mcg/dL), an adrenocorticotropic hormone (ACTH) level of 87 pg/mL (normal range, 6 to 48 pg/mL), a 24-hour urinary free cortisol level of 777 mcg/24 hours (normal range, 0 to 55 mcg/hour), and thyroid-stimulating hormone and total T4 levels within normal limits. A work-up for multiple endocrine neoplasia (MEN) type-1 (MEN-1) was negative.Computed tomography (CT) scans with contrast of the abdomen and pelvis showed no adrenal masses. A magnetic resonance imaging (MRI) scan with gadolinium of the brain and parasellar region was read as normal. A CT scan of the chest showed a mediastinal mass (Fig 1). The patient underwent an MRI and octreotide scan of the chest. The MRI scan showed an anterior heterogeneous enhancing mediastinal mass (6.0 ϫ 3.0 cm) abutting the ascending aorta, the medial segment of the left brachiocephalic vein, the upper segment of the superior vena cava, and the base of the pulmonary trunk (Fig 2A). No gross vascular invasion was identified. A left prevascular soft tissue lesion (1.9 ϫ 1.5 cm), possibly a satellite nodule or adenopathy, and right hilar adenopathy (2.0 ϫ 1.9 cm) were also revealed. The octreotide scan showed uptake in the anterior mediastinum ( Fig 2B). The patient underwent a CT-guided coaxial core biopsy of the anterior mediastinal mass. The findings were consistent with a neuroendocrine tumor, most likely a carcinoid.After careful discussion between the endocrine and pediatric hematology/oncology services, we decided to perform a transsternal resection of the tumor. The patient underwent a median sternotomy with resection of the anterior mediastinal tumor, right hilar pulmo-D I A G N O S I S I N O N C O L O G Y

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Section: Discussionmentioning

confidence: 99%

Thymic Carcinoid Presenting With Cushing's Syndrome in a 17-Year-Old Boy: A Case Report and Review of the Literature

McEvoy

Rich

New

et al. 2011

JCO

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“…Since then, this term has been widely applied to a variety of welldifferentiated neuroendocrine neoplasms at various sites, including the gastrointestinal tract, lung, and thymus (9,55,56). Duh et al (8) stated that thymic carcinoids are clinically malignant in approximately 82% of cases, whereas bronchial carcinoids are malignant in only 26% of cases.…”

Section: Discussionmentioning

confidence: 99%

Primary Neuroendocrine Carcinoma (Thymic Carcinoid) of the Thymus with Prominent Oncocytic Features: A Clinicopathologic Study of 22 Cases

Moran

Suster

2000

Modern Pathology

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Twenty-two cases of oncocytic thymic neuroendocrine carcinomas (carcinoid tumors) are presented. The patients were 17 men and 5 women between the ages of 26 and 84 years (median, 55 years). Nine were asymptomatic, and the tumor was found on routine examination; four patients presented with chest pain, two with weight loss, two with multiple endocrine neoplasia I syndrome, and one with Cushing's syndrome. Surgical resection of the mediastinal tumor was performed in all cases. The lesions were described as soft, light tan to brown, measuring from 3 to 20 cm in greatest diameter. On cut section, the tumors showed a homogeneous surface, soft consistency, and focal areas of hemorrhage. Microscopically, the lesions were characterized by nests or trabeculae of tumor cells that contained abundant granular to densely eosinophilic cytoplasm, with round to oval nuclei and in some areas prominent nucleoli. Mitotic figures ranged from 2 to 10 per 10 high-power fields; foci of comedonecrosis were seen in all cases. Immunohistochemical studies including broad spectrum keratin, CAM 5.2, chromogranin, synaptophysin, Leu-7, and p53 were performed in 12 cases. All of the tumors were strongly positive for CAM 5.2 lowmolecular-weight cytokeratin, 11 showed strong positive reaction for Leu-7, 10 for broad-spectrum keratin, 8 for chromogranin, 7 for synaptophysin, and only 1 case showed focal positive staining of the tumor cells for p53. Clinical follow-up of 14 patients showed that 10 were alive between 2 and 11 years, and 4 patients had died of tumor from 4 to 11 years after diagnosis. Patients with good clinical outcome were those whose tumors showed low mitotic activity and minimal nuclear pleomorphism, whereas those who had died of their tumors were those whose tumors were characterized by marked nuclear atypia and higher mitotic rates. Primary neuroendocrine carcinomas of the thymus are unusual neoplasms that may take the form of either moderately and well-differentiated tumors (thymic carcinoid) or poorly differentiated neoplasms (small cell neuroendocrine carcinoma of the thymus). Thymic neuroendocrine carcinomas may display a diverse spectrum of differentiation. Tumors composed predominantly of spindle cells (1, 2), with mucinous stroma (3), with divergent cell lines including mesenchymal elements and amyloid-like stroma (4 -6), and cases showing transitional areas with small cell carcinoma (7) have been described in the literature. We present a study of 22 cases of another unusual morphologic variant of primary neuroendocrine carcinoma of the thymus characterized by prominent oncocytic features. The clinicopathologic, immunohistochemical, and differential diagnostic features of these lesions are discussed. MATERIALS AND METHODSTwenty-two cases of primary thymic neuroendocrine tumors of the thymus (thymic carcinoid) with prominent oncocytic features were identified from

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“…Von den etwa 100 Thymuskarzinoiden, über die seit 1972 berichtet worden ist, hatten mindestens 40 Metastasen gesetzt. Sie betrafen, soweit ersichtlich, in 30 Fällen Männer (1,2,6,8,13,14,17,19,20,22,26,30,31,33,36,38,42,44,45) und in zehn Fällen Frauen (5,12,15,17,21,33,37,40,42,45). Dieses Verhältnis entspricht etwa der Gesamtverteilung der Thymuskarzinoide auf beide Geschlechter.…”

Section: Die Tumoren Können Hinsichtlich Des Klinischen Erscheinungsbildes In Drei Gruppen Eingeteilt Werdenunclassified

Morphologie und Klinik des Thymuskarzinoids

Wöckel

Hofmann²,

Rolle³

et al. 2008

Dtsch med Wochenschr

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A large mediastinal tumour was discovered radiologically in a 50-year-old man, with at times blood-tinged sputum and dyspnoea. Parasternal needle biopsy revealed a thymic carcinoid. After surgical excision of the tumour, which had argyrophilic foci (total weight of tumour 1,605 g), histochemical analysis demonstrated neuron-specific enolase, synaptophysin and chromogranin, as well as calcitonin (as the only hormone). For the first time in a thymic carcinoid, radioimmunoassay demonstrated growth-hormone-releasing hormone (2 micrograms/g tumour tissue). Post-operative radiotherapy (total dosage 50.5 Gy) was instituted because histological examination had shown invasion of the capsule and blood vessels. There has been no local recurrence or metastases after three years.

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Thymus Carcinoid

Cited by 26 publications

References 9 publications

Thymic Carcinoid Presenting With Cushing's Syndrome in a 17-Year-Old Boy: A Case Report and Review of the Literature

Thymic Carcinoid Presenting With Cushing's Syndrome in a 17-Year-Old Boy: A Case Report and Review of the Literature

Primary Neuroendocrine Carcinoma (Thymic Carcinoid) of the Thymus with Prominent Oncocytic Features: A Clinicopathologic Study of 22 Cases

Morphologie und Klinik des Thymuskarzinoids

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