Undifferentiated Sarcoma: Characteristics and Diagnosis

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Questions and Answers

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?

<p>Vestibular portion of the eighth nerve (A)</p>
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Which of the following is the most characteristic microscopic feature of plexiform neurofibromas?

<p>Diffuse growth within a nerve (C)</p>
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Which of the following peripheral nerve sheath tumors is most likely to be associated with symptoms related to nerve root compression?

<p>Schwannoma (C)</p>
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What is the characteristic cytologic feature of Undifferentiated Sarcoma Pleomorphic Fibroblastic Sarcoma/Pleomorphic Undifferentiated Sarcoma?

<p>Presence of bizarre multinucleate cells (D)</p>
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Which of the following is true about the chromosomal profile of Undifferentiated Sarcoma Pleomorphic Fibroblastic Sarcoma/Pleomorphic Undifferentiated Sarcoma?

<p>They have a complex karyotype with aneuploidy (B)</p>
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Where are Synovial Sarcomas typically located?

<p>Deep soft tissues around large joints of the extremities (A)</p>
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Which of the following is a characteristic feature of Synovial Sarcoma?

<p>Expression of both epithelial and mesenchymal markers (C)</p>
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What is the typical age range for the presentation of Synovial Sarcoma?

<p>20s to 40s (A)</p>
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What is the typical size range of Undifferentiated Sarcoma Pleomorphic Fibroblastic Sarcoma/Pleomorphic Undifferentiated Sarcoma?

<p>5 cm to 20 cm (B)</p>
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Which type of nerve sheath tumor is associated with a small but real risk of malignant transformation?

<p>Diffuse neurofibromas (A)</p>
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What is a unique characteristic of neurofibromas compared to schwannomas?

<p>Admixture with other cell types (B)</p>
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Which nerve sheath tumor may appear circumscribed?

<p>Localized cutaneous neurofibromas (C)</p>
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What is the unique feature of diffuse neurofibromas in terms of growth pattern within the skin?

<p>Extensive infiltrative pattern (A)</p>
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Which type of nerve sheath tumor involves multiple fascicles of individual affected nerves?

<p>Plexiform neurofibromas (C)</p>
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What is the genetic basis of Neurofibromatosis-1 (NF1)?

<p>Linked to chromosome 17 (17q) (A)</p>
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Which of the following is NOT a common metastatic site for cancers?

<p>Brain (A)</p>
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What is the typical 5-year survival rate for cancers that have metastasized?

<p>50% (B)</p>
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Which of the following is NOT a tumor-like lesion described in the text?

<p>Osteosarcoma (B)</p>
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What is the typical size of a ganglion cyst?

<p>Less than 1.5 cm in diameter (A)</p>
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Where are Tenosynovial Giant Cell Tumors most commonly found?

<p>Knee (B)</p>
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What is the typical age range for patients with Tenosynovial Giant Cell Tumor?

<p>20s-30s (A)</p>
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Which statement about rhabdomyosarcoma is correct?

<p>The alveolar variant is characterized by the $t(2;13)$ PAX3-FKHR fusion. (A)</p>
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Which of the following is true about sarcoma botryoides?

<p>It is an embryonal variant of rhabdomyosarcoma arising near mucosal surfaces. (C)</p>
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Which of the following statements about leiomyomas is incorrect?

<p>They are malignant tumors. (A)</p>
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Which of the following is a diagnostic feature of rhabdomyosarcoma?

<p>Presence of rhabdomyoblasts with granular eosinophilic cytoplasm. (B)</p>
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Which of the following statements about leiomyosarcomas is correct?

<p>Histologically, they show spindle cells in interwoven fascicles. (C)</p>
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Which of the following immunohistochemical markers is useful in confirming the smooth muscle origin of a tumor?

<p>Desmin and h-caldesmon (C)</p>
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