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Presented by Dr. Justin Bishop and prepared by Dr. Jason Kern
50 year old man with a laryngeal mass.
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1. Question
50 year old man with a laryngeal mass.
Correct
Answer: Rhabdomyoma
Description/Discussion:
The tumor consists of an unencapsulated sheet-like proliferation of large polygonal cells with granular brightly eosinophilic cytoplasm. Some cells demonstrate cytoplasmic vacuolization resulting in the appearance of fibrillar processes (so-called “spider cells”). Cross striations or cytoplasmic crystals can been seen in some tumor cells as well. There is no significant nuclear atypia, mitoses, or necrosis. Immunostaining showed that the tumor was positive for desmin and negative for myogenin. The findings are consistent with an adult-type rhabdomyoma.The term “rhabdomyoma” is confusing because it may refer to many different tumor types, including PEComas involving the heart (often seen in patients with tuberous sclerosis) and certain hamartomatous proliferations of the skin and soft tissue. “True” rhabdomyomas are subdivided into fetal and adult types. The fetal type may be difficult to differentiate from rhabdomyosarcoma, while the adult type is most likely to be confused with granular cell tumor, paraganglioma, or hibernoma. Adult type rhabdomyomas occur in adults with a peak in the 6th decade, and they involve the head and neck particularly larynx, pharynx, and parapharygneal space. Because they are comprised of mature skeletal muscle, it is important to remember that myogenin is usually negative (as it is in normal skeletal muscle). Desmin is consistently, strongly positive. Adult type rhabdomyoma is benign, with a low risk of recurrence following resection.
Incorrect
Answer: Rhabdomyoma
Description/Discussion:
The tumor consists of an unencapsulated sheet-like proliferation of large polygonal cells with granular brightly eosinophilic cytoplasm. Some cells demonstrate cytoplasmic vacuolization resulting in the appearance of fibrillar processes (so-called “spider cells”). Cross striations or cytoplasmic crystals can been seen in some tumor cells as well. There is no significant nuclear atypia, mitoses, or necrosis. Immunostaining showed that the tumor was positive for desmin and negative for myogenin. The findings are consistent with an adult-type rhabdomyoma.The term “rhabdomyoma” is confusing because it may refer to many different tumor types, including PEComas involving the heart (often seen in patients with tuberous sclerosis) and certain hamartomatous proliferations of the skin and soft tissue. “True” rhabdomyomas are subdivided into fetal and adult types. The fetal type may be difficult to differentiate from rhabdomyosarcoma, while the adult type is most likely to be confused with granular cell tumor, paraganglioma, or hibernoma. Adult type rhabdomyomas occur in adults with a peak in the 6th decade, and they involve the head and neck particularly larynx, pharynx, and parapharygneal space. Because they are comprised of mature skeletal muscle, it is important to remember that myogenin is usually negative (as it is in normal skeletal muscle). Desmin is consistently, strongly positive. Adult type rhabdomyoma is benign, with a low risk of recurrence following resection.
