This document discusses a case of pheochromocytoma, a rare tumor arising in the adrenal medulla that secretes catecholamines. It describes the gross and microscopic pathology of the tumor, which appeared as a large grey-tan mass in the adrenal gland of a patient with episodic hypertension. Common symptoms of pheochromocytoma are also outlined, including severe headaches, sweating, and heart palpitations. The tumor is discussed in the context of specific hereditary conditions and syndromes that can cause pheochromocytoma such as MEN1/2 and von Hippel-Lindau disease.
This document discusses a case of pheochromocytoma, a rare tumor arising in the adrenal medulla that secretes catecholamines. It describes the gross and microscopic pathology of the tumor, which appeared as a large grey-tan mass in the adrenal gland of a patient with episodic hypertension. Common symptoms of pheochromocytoma are also outlined, including severe headaches, sweating, and heart palpitations. The tumor is discussed in the context of specific hereditary conditions and syndromes that can cause pheochromocytoma such as MEN1/2 and von Hippel-Lindau disease.
This document discusses a case of pheochromocytoma, a rare tumor arising in the adrenal medulla that secretes catecholamines. It describes the gross and microscopic pathology of the tumor, which appeared as a large grey-tan mass in the adrenal gland of a patient with episodic hypertension. Common symptoms of pheochromocytoma are also outlined, including severe headaches, sweating, and heart palpitations. The tumor is discussed in the context of specific hereditary conditions and syndromes that can cause pheochromocytoma such as MEN1/2 and von Hippel-Lindau disease.
This document discusses a case of pheochromocytoma, a rare tumor arising in the adrenal medulla that secretes catecholamines. It describes the gross and microscopic pathology of the tumor, which appeared as a large grey-tan mass in the adrenal gland of a patient with episodic hypertension. Common symptoms of pheochromocytoma are also outlined, including severe headaches, sweating, and heart palpitations. The tumor is discussed in the context of specific hereditary conditions and syndromes that can cause pheochromocytoma such as MEN1/2 and von Hippel-Lindau disease.
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Path Lab
Name: Onyedika Egbujo
No: #671 Topic: Pheochromocytoma
Identify the Tissue with reason
This large adrenal neoplasm has been sectioned in half. Note the grey-tan color of the tumor compared to the yellow cortex stretched around it and a small remnant of remaining adrenal at the lower right. This patient had episodic hypertension. This is a tumor arising in the adrenal medulla--a pheochromocytoma. Describe gross morphology changes Varies from small, circumscribed to large, hemorrhagic and necrotic (1g to 4 kg) Mean 7 cm / 200 g Small tumors have rim of compressed adrenal gland Lobulated, yellow-red-brown Familial tumors are bilateral and multicentric, and adjacent medulla may appear hyperplastic Chromaffin reaction: Fresh tumor turns dark brown if add potassium dichromate at pH 5-6 (Zenkers)
Describe histopathology changes Zellballen (small nests or alveolar pattern), trabecular or solid patterns of polygonal / spindle shaped cells in rich vascular network Cells have finely granular basophilic or amphophilic cytoplasm Intracytoplasmic hyaline globules Round / oval nuclei with prominent nucleolus and variable inclusion-like structures. May have marked pleomorphism Capsular and vascular invasion common in benign behaving tumors Nests outlined by sustentacular cells (cannot see in H&E but S100+)
Amyloid also common. Rare / no mitotic figures May be mixed with neuroblastoma, ganglioneuroma, ganglioneuroblastoma, cortical adenoma, spindle cell sarcoma Unusual morphological features: o Coexisting cortical hyperplasia, vacuolar degeneration of tumor cells, presence of pheochromoblasts (small cells), ganglion-like cells, calcospherites, melanin pigmentation, insular growth pattern, brown fat
List possible etiology/ risk factors Probably >10% of cases; usually younger age of onset (10-20 years vs. 40- 60 years), bilateral (70% vs. 10% in sporadic), multicentric, associated with other neoplasms, male gender MEN 1: o Pituitary tumors, parathyroid hyperplasia or adenoma, pancreatic hyperplasia or adenoma and carcinoid tumors MEN 2a/2: o Sipple first described in 1961 o Autosomal dominant with high penetrance, 30-50% have pheochromocytoma, all have medullary thyroid carcinoma and 10- 15% have parathyroid hyperplasia o Due to mutation in RET proto-oncogene MEN 2b/3: o MEN2a signs / symptoms plus mucosal neuromas and ganglioneuromas o Autosomal dominant or sporadic o May lack parathyroid hyperplasia Sturge-Weber: o Cavernous hemangiomas (port-wine spots) of trigeminal nerve (cranial nerve V), pheochromocytoma von Hippel Lindau: o 10-20% of patients o Cysts of kidney, liver and epididymis o Renal cell carcinoma (clear cell type), pheochromocytoma, angiomas and cerebellar hemangioblastoma von Recklinghausen (neurofibromatosis type 1): o 1-5% of patients o Neurofibromatosis, schwannoma, meningioma, glioma and pheochromocytoma
o Composite tumors with neuroblastoma, ganglioneuroma or ganglioneuroblastoma may be associated with neurofibromatosis 1 Describe the pathogenic mechanism The clinical manifestations of a pheochromocytoma result from excessive catecholamine secretion by the tumor. Secretion may occur either intermittently or continuously. Catecholamines typically secreted are norepinephrine and epinephrine; some tumors produce dopamine. What is the pathology or diagnosis Episodic hypertension Causes surgically correctable hypertension (also aldosterone-secreting tumors, renal artery stenosis) Called 10% tumor: 10% bilateral (probably higher), 10% outside adrenal medulla, 10% metastasize (probably higher), 10% in children Extra-adrenal tumors secrete only norepinephrine, have 20% malignancy rate In children, usually extra-adrenal, bilateral and associated with MEN 2a/2b Metastases usually to lymph nodes, bone (ribs, spine), liver and lung; occasionally to brain, diaphragm, ileum, kidney, pancreas, peritoneum, pleura, spleen, stomach Catecholamine cardiomyopathy: o Myocardial instability, ventricular arrhythmias o Due to ischemic damage from vasoconstriction of coronaries or direct toxicity o Cardiac biopsy shows myocytolysis, interstitial fibrosis and mononuclear inflammation Diagnosis: o Increased urinary excretion of catecholamines or metabolites (vanilllylmandelic acid-VMA or total metanephrines) o Elevated serum chromogranin A levels o Clonidine test List symptoms/signs The symptoms of phaeochromocytoma tend to be unpredictable you may suddenly get an attack lasting 15-20 minutes. The three typical symptoms are: severe headaches sweating heart palpitations Other possible symptoms are: high blood pressure feeling irritable, nervous or anxious having problems sleeping tremors stomach pains weakness weight loss The attacks may become more frequent, longer and more severe as the tumour grows.
However, some people with phaeochromocytoma never develop symptoms, and their condition is only discovered during tests for another problem. List other systems affected/complicatio ns Hypertension Hypertensive encephalopathy Myocarditis Signs and symptoms of myocardial infarction Dilated cardiomyopathy Pulmonary edema: Either of cardiac or noncardiac origin In pregnancy: maternal and fetal mortality rates of 48% and 55%, respectively. List relevant/additional info Criteria for malignancy Most reliable is presence of metastases (lymph nodes, liver, lung, bone) Malignant cases have MIB1 positive cell rate of 2-3% or more. PASS score (Pheochromocytoma of the Adrenal gland Scaled Score): o Benign if 0-3 points, potential for malignancy if 4+ points; each feature present gets points added. o 1 point: vascular invasion, capsular invasion, profound nuclear pleomorphism or hyperchromasia o 2 points: invasion of periadrenal adipose tissue, large nests or diffuse growth, focal or confluent necrosis, high cellularity, tumor cell spindling, cellular monotony, 4+ mitotic figures per 10 high power fields, atypical mitotic figures Write a clinical vignette A 50-year-old man has episodic headaches for 3 months. On physical examination his blood pressure is 185/110 mm Hg, with no other remarkable findings. Laboratory studies show sodium 145 mmol/L, potassium 4.3 mmol/L, chloride 103 mmol/L, C02 26 mmol/L, glucose 91 mg/dL, and creatinine 1.3 mg/dL. An abdominal CT scan shows a 7 cm left adrenal mass. During surgery, as the left adrenal gland is removed, there a marked rise in blood pressure. Which of the following laboratory test findings most likely explains his findings? A Decreased serum cortisol B Decreased urinary homovanillic acid C Increased serum ACTH D Increased urinary free catecholamines E Elevated serum ANCA List 3 important features that might be tested Risk factors, pathologic mechanism and morphological features.