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Questions and Answers
What is the characteristic histological feature of the most well-known variant of malignant peripheral nerve sheath tumor?
What is the characteristic histological feature of the most well-known variant of malignant peripheral nerve sheath tumor?
- Presence of mucin-producing glands
- Lack of immunoreactivity for myoglobin
- Presence of rhabdomyosarcomatous differentiation (correct)
- Absence of necrotic areas
Which immunohistochemical marker is strongly positive in epithelioid malignant peripheral nerve sheath tumor?
Which immunohistochemical marker is strongly positive in epithelioid malignant peripheral nerve sheath tumor?
- Myoglobin
- Desmin
- S-100 protein (correct)
- Cytokeratin
What is the term used to describe a malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation?
What is the term used to describe a malignant peripheral nerve sheath tumor with rhabdomyosarcomatous differentiation?
- Neurofibroma
- Epithelioid MPNST
- Malignant triton tumor (correct)
- Angiosarcoma
What is the significance of recognizing areas of MPNST in a tumor with divergent differentiation?
What is the significance of recognizing areas of MPNST in a tumor with divergent differentiation?
What is a differential diagnosis to consider in a tumor with divergent differentiation, if areas of MPNST are not recognizable?
What is a differential diagnosis to consider in a tumor with divergent differentiation, if areas of MPNST are not recognizable?
What is a histological feature of epithelioid malignant peripheral nerve sheath tumor?
What is a histological feature of epithelioid malignant peripheral nerve sheath tumor?
What is a characteristic feature of malignant peripheral nerve sheath tumor with angiosarcomatous differentiation?
What is a characteristic feature of malignant peripheral nerve sheath tumor with angiosarcomatous differentiation?
What is a prognostic factor to consider in a patient with malignant peripheral nerve sheath tumor?
What is a prognostic factor to consider in a patient with malignant peripheral nerve sheath tumor?
What is a characteristic feature of psammomatous melanotic schwannoma?
What is a characteristic feature of psammomatous melanotic schwannoma?
Which of the following is a differential diagnosis for melanotic schwannoma?
Which of the following is a differential diagnosis for melanotic schwannoma?
What is a characteristic location for schwannomas with a microcystic/reticular appearance?
What is a characteristic location for schwannomas with a microcystic/reticular appearance?
What is a rare feature of otherwise typical schwannomas or those with epithelioid features?
What is a rare feature of otherwise typical schwannomas or those with epithelioid features?
What is the prognosis of psammomatous melanotic schwannoma?
What is the prognosis of psammomatous melanotic schwannoma?
What is a characteristic feature of the cells in a granular cell tumor?
What is a characteristic feature of the cells in a granular cell tumor?
Which of the following immunohistochemical markers is typically positive in granular cell tumors?
Which of the following immunohistochemical markers is typically positive in granular cell tumors?
Which complex is psammomatous melanotic schwannoma a component of?
Which complex is psammomatous melanotic schwannoma a component of?
What is a characteristic of schwannoma by electron microscopy?
What is a characteristic of schwannoma by electron microscopy?
What is the gross appearance of a neurofibroma?
What is the gross appearance of a neurofibroma?
Which of the following is a differential diagnosis for granular cell tumors?
Which of the following is a differential diagnosis for granular cell tumors?
What is a characteristic feature of neurofibroma?
What is a characteristic feature of neurofibroma?
What is the significance of S-100 protein positivity in granular cell tumors?
What is the significance of S-100 protein positivity in granular cell tumors?
What is the term for the morphology of some granular cell tumors that have focal granular changes?
What is the term for the morphology of some granular cell tumors that have focal granular changes?
What is the name of the condition associated with plexiform neurofibromas?
What is the name of the condition associated with plexiform neurofibromas?
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.
