Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0153690 (bone metastases)
 6,382 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Pheochromocytoma is a cause of hypertension that frequently can be cured by surgery. The aim of this paper, based on 5 cases of pheochromocytoma, is to relate our experience in diagnosis and treatment in this pathology. In four of 5 patients with pheochromocytoma we observed unusual characteristics of the disease. Association with neurofibromatosis in one case, with rheumatic mitral regurgitation in another; and in a third case the tumor was malignant. One patient had catecholamine-mediated electrocardiographic changes which disappeared with treatment. Since symptoms of adrenergic hyperactivity were present in all cases, the rise in the levels of vanilmandelic acid and urinary metanephrines were useful in confirming the diagnosis. Computed tomography and I-131 meta-benzylguanidine for radioisotopic imaging, displayed not only all tumoral masses but also bone metastases in the malignant case. During the follow-up period, from the sixth month to the fourth year after surgery, four patients were asymptomatic, and have normal urinary catecholamine metabolite levels. The patient with a malignant form of pheochromocytoma continued to show elevated catecholamines release and remained hypertensive in spite of adrenal mass resection.
 ...
PMID:[Pheochromocytoma. Its diagnostic and therapeutic characteristics]. 134 Jul 39

Adrenal metastases of the papillary thyroid carcinoma (PTC) are very rare. We report one case. A 63-year-old woman had undergone 15 years earlier left lobo-ishmectomy for a papillary thyroid cancer (PTC) and 7 years earlier right adrenalectomy for a tumor. Histologic examination showed a benign cortical tumor. In 1999, when the patient was admitted for worsening of glycemic control, a recurrence of the adrenal mass was detected. According to the hormone evaluation it was a non-functional tumor. Adrenalectomy was performed in June 2002 because the patient had initially declined surgery. Histologic examination and thyroglobulin immunochemistry identified metastatic PTC. Re-reading the histology slide of the first adrenalectomy agreed with the diagnosis. Thyroidectomy was completed in March 2003. Although iodine-131 therapy and thyroxine treatment were given, bone metastases were detected in August 2004. PTC usually spreads to the cervical and mediastinal lymph nodes. Distant spread may occur to bone or lung, but exceptionally to the adrenal gland. The adrenal localization is often associated with lung or bone metastasis. In our patient, the adrenal metastasis remained isolated for many years. It has been reported that survival rate decreases considerably after appearance of a distant metastasis. Although given delayed radical treatment, our patient remained alive 13 years after.
 ...
PMID:[Adrenal metastasis of a papillary thyroid cancer]. 1707 45

Catecholamine-producing tumors may arise in the adrenal medulla (pheochromocytomas) or in extraadrenal chromaffin cells (secreting paragangliomas). Their prevalence is about 0.1% in patients with hypertension and 4% in patients with a fortuitously discovered adrenal mass. An increase in the production of catecholamines causes symptoms (mainly headaches, palpitations and excess sweating) and signs (mainly hypertension, weight loss and diabetes) reflecting the effects of epinephrine and norepinephrine on alpha- and beta-adrenergic receptors. Catecholamine-producing tumors mimic paroxysmal conditions with hypertension and/or cardiac rhythm disorders, including panic attacks, in which sympathetic activation linked to anxiety reproduces the same signs and symptoms. These tumors may be sporadic or part of any of several genetic diseases: familial pheochromocytoma-paraganglioma syndromes, multiple endocrine neoplasia type 2, neurofibromatosis 1 and von Hippel-Lindau disease. Familial cases are diagnosed earlier and are more frequently bilateral and recurring than sporadic cases. The most specific and sensitive diagnostic test for the tumor is the determination of plasma or urinary metanephrines. The tumor can be located by computed tomography, magnetic resonance imaging and metaiodobenzylguanidine scintigraphy. Treatment requires resection of the tumor, generally by laparoscopic surgery. About 10% of tumors are malignant either at first operation or during follow-up, malignancy being diagnosed by the presence of lymph node, visceral or bone metastases. Recurrences and malignancy are more frequent in cases with large or extraadrenal tumors. Patients, especially those with familial or extraadrenal tumors, should be followed-up indefinitely.
 ...
PMID:Pheochromocytomas and secreting paragangliomas. 1715 52

Among adrenal incidentalomas, pheochromocytomas are rare. Malignant pheochromocytoma is even less common, and it typically presents with classic hormonal symptoms, such as palpitations, labile blood pressures, and headaches. Bony metastasis usually occurs late in disease, but we report an unusual case of incapacitating bony pain as the initial presentation of malignant pheochromocytoma. Our patient is a 70-year-old woman with neurofibromatosis type 1 and a history of primary hyperparathyroidism, who tested negative for the ret mutation. She came to medical attention with chest pain and palpitations and was incidentally found to have an adrenal mass. Serum and urine testing was consistent with pheochromocytoma. Her blood pressure was easily controlled as she awaited elective adrenalectomy; however, she quickly developed severe, diffuse bony pain. She represented with hypercalcemia, spontaneous fractures, and incapacitating pain that required such high doses of pain medications that she had to be intubated. Further imaging and bone marrow biopsy confirmed metastatic neuroendocrine tumor. She received one round of chemotherapy with no change in her bony pain, which was her primary complaint. Unfortunately, her treatment options were limited by the heavy sedation required for comfort, and in the end, it was her bony pain rather than hormonal symptoms that made her disease untreatable.
 ...
PMID:Malignant pheochromocytoma presenting as incapacitating bony pain. 2267 44

In the treatment of hepatocellular carcinoma, atypical, off guideline multidisciplinary approaches are sometimes effective. A 70-year-old man was diagnosed with multiple hepatocellular carcinomas, multiple bone metastases, and a right adrenal metastasis. Sunitinib was started and the primary hepatic lesions and bone metastases disappeared. However, his adrenal metastasis worsened. Sorafenib, radiotherapy, and some investigational agents were administered, but the adrenal metastasis did not respond. There were no other new lesions except the adrenal lesion 4 years after the initial treatment, so we decided to perform a resection. In the left half lateral decubitus position, the adrenal mass was removed with right thoracolaparotomy. After the surgery, his tumor markers quickly returned to normal. Seven years after the initial treatment(2 years and 4 months after the last surgery), he is alive without any recurrence. Multidisciplinary treatment with chemotherapy, radiotherapy, and surgery may result in long term survival even for patients with advanced hepatocellular carcinoma with multiple extra-hepatic lesions.
 ...
PMID:[Resection of Adrenal Metastasis after Chemotherapy and Radiation Therapy for a Patient with Stage IV B Hepatocellular Carcinoma]. 2813 68