Undifferentiated Sarcoma: Characteristics and Diagnosis

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Which type of peripheral nerve sheath tumor is most commonly associated with neurofibromatosis type 1 (NF1)?

Malignant peripheral nerve sheath tumor

Plexiform neurofibroma (correct)

Schwannoma

Localized cutaneous neurofibroma

What is the key microscopic feature that distinguishes schwannomas from neurofibromas?

The presence of a dense cellular area (Antoni A) and a loose hypocellular area (Antoni B)

The presence of nuclear palisading and Verocay bodies (correct)

The presence of axons within the tumor

The presence of a well-defined capsule around the tumor

Which of the following statements about the anatomical origin of peripheral nerve sheath tumors is correct?

Malignant peripheral nerve sheath tumors can arise from pre-existing benign peripheral nerve sheath tumors (correct)

Neurofibromas always arise from the endoneurium of a nerve

All peripheral nerve sheath tumors originate from the nerve root

Schwannomas always arise from the perineurium of a nerve

What is the most common cranial nerve affected by schwannomas?

Vestibular portion of the eighth nerve Signup and view all the answers

Which of the following is the most characteristic microscopic feature of plexiform neurofibromas?

Diffuse growth within a nerve Signup and view all the answers

Which of the following peripheral nerve sheath tumors is most likely to be associated with symptoms related to nerve root compression?

Schwannoma Signup and view all the answers

What is the characteristic cytologic feature of Undifferentiated Sarcoma Pleomorphic Fibroblastic Sarcoma/Pleomorphic Undifferentiated Sarcoma?

Presence of bizarre multinucleate cells Signup and view all the answers

Which of the following is true about the chromosomal profile of Undifferentiated Sarcoma Pleomorphic Fibroblastic Sarcoma/Pleomorphic Undifferentiated Sarcoma?

They have a complex karyotype with aneuploidy Signup and view all the answers

Where are Synovial Sarcomas typically located?

Deep soft tissues around large joints of the extremities Signup and view all the answers

Which of the following is a characteristic feature of Synovial Sarcoma?

Expression of both epithelial and mesenchymal markers Signup and view all the answers

What is the typical age range for the presentation of Synovial Sarcoma?

20s to 40s Signup and view all the answers

What is the typical size range of Undifferentiated Sarcoma Pleomorphic Fibroblastic Sarcoma/Pleomorphic Undifferentiated Sarcoma?

5 cm to 20 cm Signup and view all the answers

Which type of nerve sheath tumor is associated with a small but real risk of malignant transformation?

Diffuse neurofibromas Signup and view all the answers

What is a unique characteristic of neurofibromas compared to schwannomas?

Admixture with other cell types Signup and view all the answers

Which nerve sheath tumor may appear circumscribed?

Localized cutaneous neurofibromas Signup and view all the answers

What is the unique feature of diffuse neurofibromas in terms of growth pattern within the skin?

Extensive infiltrative pattern Signup and view all the answers

Which type of nerve sheath tumor involves multiple fascicles of individual affected nerves?

Plexiform neurofibromas Signup and view all the answers

What is the genetic basis of Neurofibromatosis-1 (NF1)?

Linked to chromosome 17 (17q) Signup and view all the answers

Which of the following is NOT a common metastatic site for cancers?

Brain Signup and view all the answers

What is the typical 5-year survival rate for cancers that have metastasized?

50% Signup and view all the answers

Which of the following is NOT a tumor-like lesion described in the text?

Osteosarcoma Signup and view all the answers

What is the typical size of a ganglion cyst?

Less than 1.5 cm in diameter Signup and view all the answers

Where are Tenosynovial Giant Cell Tumors most commonly found?

Knee Signup and view all the answers

What is the typical age range for patients with Tenosynovial Giant Cell Tumor?

20s-30s Signup and view all the answers

Which statement about rhabdomyosarcoma is correct?

The alveolar variant is characterized by the $t(2;13)$ PAX3-FKHR fusion. Signup and view all the answers

Which of the following is true about sarcoma botryoides?

It is an embryonal variant of rhabdomyosarcoma arising near mucosal surfaces. Signup and view all the answers

Which of the following statements about leiomyomas is incorrect?

They are malignant tumors. Signup and view all the answers

Which of the following is a diagnostic feature of rhabdomyosarcoma?

Presence of rhabdomyoblasts with granular eosinophilic cytoplasm. Signup and view all the answers

Which of the following statements about leiomyosarcomas is correct?

Histologically, they show spindle cells in interwoven fascicles. Signup and view all the answers

Which of the following immunohistochemical markers is useful in confirming the smooth muscle origin of a tumor?

Desmin and h-caldesmon Signup and view all the answers