Questions and Answers
What is the characteristic histological feature of the most well-known variant of malignant peripheral nerve sheath tumor?
Which immunohistochemical marker is strongly positive in epithelioid malignant peripheral nerve sheath tumor?
What is the term used to describe a malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation?
What is the significance of recognizing areas of MPNST in a tumor with divergent differentiation?
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What is a differential diagnosis to consider in a tumor with divergent differentiation, if areas of MPNST are not recognizable?
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What is a histological feature of epithelioid malignant peripheral nerve sheath tumor?
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What is a characteristic feature of malignant peripheral nerve sheath tumor with angiosarcomatous differentiation?
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What is a prognostic factor to consider in a patient with malignant peripheral nerve sheath tumor?
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What is a characteristic feature of psammomatous melanotic schwannoma?
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Which of the following is a differential diagnosis for melanotic schwannoma?
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What is a characteristic location for schwannomas with a microcystic/reticular appearance?
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What is a rare feature of otherwise typical schwannomas or those with epithelioid features?
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What is the prognosis of psammomatous melanotic schwannoma?
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What is a characteristic feature of the cells in a granular cell tumor?
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Which of the following immunohistochemical markers is typically positive in granular cell tumors?
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Which complex is psammomatous melanotic schwannoma a component of?
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What is a characteristic of schwannoma by electron microscopy?
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What is the gross appearance of a neurofibroma?
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Which of the following is a differential diagnosis for granular cell tumors?
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What is a characteristic feature of neurofibroma?
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What is the significance of S-100 protein positivity in granular cell tumors?
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What is the term for the morphology of some granular cell tumors that have focal granular changes?
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What is the name of the condition associated with plexiform neurofibromas?
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Study Notes
Differentiation in Malignant Peripheral Nerve Sheath Tumors
- Malignant peripheral nerve sheath tumors can have divergent differentiation, such as angiosarcomatous and mucin-producing glands
- Rhabdomyosarcomatous differentiation is characterized by the presence of rhabdomyosarcoma-like cells
- This variant is often referred to as a malignant triton tumor
- Diagnosis requires the presence of areas of recognizable malignant peripheral nerve sheath tumor
Epithelioid Malignant Peripheral Nerve Sheath Tumors
- Epithelioid malignant peripheral nerve sheath tumors exhibit strong S-100 protein immunoreactivity
- They can have a lipoblastic component, accompanied by cells with signet ring–like features
- Some epithelioid schwannomas can contain foci of small, round, hyperchromatic Schwann cells with scanty cytoplasm, simulating neuroblastoma
Psammomatous Melanotic Schwannoma
- Psammomatous melanotic schwannoma is a distinctive type of peripheral nerve sheath tumor
- It occurs as a component of the Carney complex
- It is characterized microscopically by the presence of melanin pigmentation and the deposition of psammoma bodies
- It is regarded as a low-grade malignancy due to its tendency for local recurrence and ability to metastasize
Granular Cell Tumors
- Granular cell tumors are characterized by the presence of innumerable fine cytoplasmic granules and scattered larger eosinophilic globules
- They can be challenging to diagnose and may be mistaken for other tumors
- The precise line of differentiation of these rare tumors remains obscure
- Granular cell change may also be found in a number of other unrelated tumors
Neurofibroma
- The gross, microscopic, and ultrastructural features of neurofibroma are distinct from those of schwannoma
- The gross appearance of neurofibroma varies greatly from lesion to lesion
- It is important to distinguish neurofibroma from schwannoma, despite the fact that in some instances the differential diagnosis may be difficult or that in isolated cases features of both lesions may coexist.
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Description
This quiz is about the characteristics and appearance of granular cell tumors, including their cellular structure and dermal permeation. It includes figures and descriptions of the tumors.