Neoplasia: haemangioblastoma in von Hippel-Lindau disease: practice question

A Pathology question in the style of the NEET PG paper. Answer it first, then read why the right option is right and why each of the others is wrong.

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Pathology · Neoplasia: haemangioblastoma in von Hippel-Lindau disease

Sample item

A 32-year-old man with a family history of renal cell carcinoma and retinal angiomas has a cerebellar cystic lesion with a mural nodule. Histology of the nodule shows foamy stromal cells in a rich capillary network. Which laboratory abnormality may be expected?

Original practice item, not a recalled exam question. Nothing here is recorded.

The answer and the reasoning

C. Polycythaemia

Haemangioblastoma is the characteristic CNS tumour of von Hippel-Lindau disease. The stromal cells can secrete erythropoietin, leading to secondary polycythaemia, which is also a feature of renal cell carcinoma in the same syndrome. Loss of VHL stabilises HIF and increases VEGF and erythropoietin production.

Option by option

  • A. Hypoglycaemia

    Hypoglycaemia occurs with insulinoma or large mesenchymal tumours making IGF-2.

  • B. Hypercalcaemia

    Hypercalcaemia from PTHrP is typical of squamous cancers, not haemangioblastoma.

  • C. Polycythaemia (correct)

    Correct. Erythropoietin production by the tumour causes polycythaemia.

  • D. Hyponatraemia

    Hyponatraemia from ADH secretion is typical of small cell lung carcinoma.

Source note: Original NextPrep sample item — pending expert review. This item is original practice material awaiting independent expert review, so confirm important facts in a standard textbook.

More Pathology questions

Questions about this Pathology question

What is the answer to this Pathology question?

The best answer is C, Polycythaemia. Haemangioblastoma is the characteristic CNS tumour of von Hippel-Lindau disease.

Why is "Hypoglycaemia" not the answer?

Hypoglycaemia occurs with insulinoma or large mesenchymal tumours making IGF-2.

Why is "Hypercalcaemia" not the answer?

Hypercalcaemia from PTHrP is typical of squamous cancers, not haemangioblastoma.

Why is "Hyponatraemia" not the answer?

Hyponatraemia from ADH secretion is typical of small cell lung carcinoma.

What concept does this question test?

It tests Neoplasia: haemangioblastoma in von Hippel-Lindau disease in Pathology. The stromal cells can secrete erythropoietin, leading to secondary polycythaemia, which is also a feature of renal cell carcinoma in the same syndrome. Loss of VHL stabilises HIF and increases VEGF and erythropoietin production.

How hard is this question?

We tag it as a multi-step question: it asks you to combine more than one idea. The tag is our own editorial judgement, not an official difficulty rating, so read the reason for every option, not only the answer.

How should I study Neoplasia: haemangioblastoma in von Hippel-Lindau disease?

Learn each disease as a bundle: cause, key histology, markers, and the clinical hallmark. Then use the flashcards and the other Pathology questions on this site to test what stuck.

Is this a real NEET PG question?

No. It is an original practice item (editorial status: sample, version 1). NextPrep does not publish recalled or leaked questions, and independent faculty review of this item has not been completed yet, so confirm important facts in a standard textbook.