Multiple endocrine neoplasm type III. Report of a case.

Contact Person: Alberto G Pizarro (rediegal@homonet.com.mx)

Results

Pathological report:

At autopsy we identified multiple neurofibromas (more 100) in skin of head, chest, abdomen and extremities and spinal cord with size of 1 to 3 cm. Figure 3 and figure 4.

Fig.3

Fig.4

Micro: hyperplasia of schwann cells. Figure 5, figure 6 ;

Fig.5

Fig.6

Stomach with nodules submucoses of 1 cm. Micro: hiperplasia of schwann cells figure 7; Heart weighted 320 gr with left hiperthrophy and subendocardial fibrosis. No thrombosis in coronaries and pulmonary arteries. Micro: coagulative necrosis of focal cells. Figure 8; Lungs with edema, hemorrhagies and macrophages.

Fig.7

Fig.8

One right central adrenal neoplam with size of 6x5x4 cm and weight 30 gr., vascularized hemorrhagies and necrotical Figure 9 and figure 10.

Fig.9

Fig.10

With capsule without metastasis or invasion Figure 10.

The cells were polyhedral or cuboidal with basophil and finelly granular or vacuolated cytoplasm Figure 11, figure 12. There is the presence of intranuclear and intracytoplasmic "Inclusion" Figure 13.

Fig.11	Fig.12	Fig13

There is hyperplasia, mitosis and nuclear pleomorfism. Reported as Pheochromocytoma.

One tumor in paraaortic was reporte as paraganglioma. Figure 14 and 15.

Fig.14

Fig.15

Two parathyroid glands with hyperplasia of chief cells with 2 gr.

One tumor en ovary reporte as benign thecofibroma.

Hepatomegaly with steatosis.

Final Diagnosis:

1. Multiple endocrin neoplams (MEA type III or IIb )

1. Systemic neurofibromatosis: skin, spinal cord, stomach.
2. Neurofibrosarcoma of right thigh.
3. Adrenal Pheochromocytoma.
4. AorticParaganglioma .
5. Pharathyroid hyperplasia (2)

1. Secundary Arterial Hypertension

1. elevated catecholamines.
2. Left ventricular hypertrophy
3. Focal necrosis cells.
4. Cardiac arrest.

1. Ovary thecofibroma .
2. Steatosis of liver.
   

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