20 - 121: NEOPLASIAS AND HYPERPLASIAS OF MUSCULAR AND NEURAL ORIGIN Flashcards
This is a major criterion in the diagnosis of hereditary leiomyomatosis and renal cell carcinoma (HLRCC).
a. Multiple cutaneous leiomyomata with at least 1 biopsy proven/ histologically confirmed
b. Solitary cutaneous leiomyoma and family history of HLRCC
c. Symptoms may be paroxysmal with worsening by light touch, temperature, or hormonal change
d. Positive germline FH-mutation test
A
This is a minor criterion in the diagnosis of hereditary leiomyomatosis and renal cell carcinoma (HLRCC).
a. Multiple cutaneous leiomyomata with at least 1 biopsy proven/ histologically confirmed
b. Solitary cutaneous leiomyoma and family history of HLRCC
c. Symptoms may be paroxysmal with worsening by light touch, temperature, or hormonal change
d. Positive germline FH-mutation test
Answer: B
This is required in the definitive diagnosis of hereditary leiomyomatosis and renal cell carcinoma .
a. Multiple cutaneous leiomyomata with at least 1 biopsy proven/ histologically confirmed
b. Solitary cutaneous leiomyoma and family history of HLRCC
c. Symptoms may be paroxysmal with worsening by light touch, temperature, or hormonal change
d. Positive germline FH-mutation test
Answer: D
This tumor presents with a firm nodule with hyperpigmentation and erythema of the involved skin, most commonly in hair-bearing areas of lower extremities, scalp, and trunk. It may develop as secondary malignancy in the setting of hereditary retinoblastoma, alterations of the retinoblastoma 1 (Rb1) locus
A. Leiomyoma
B. Angioleiomyoma
C. Leiomyosarcoma
D. Smooth muscle hamartoma
C. Leiomyosarcoma
Small papules may be detectable within the lesions and are sensitive to transient piloerection following rubbing, as seen in which sign?
Pseudo-Darier sign
Pseudo darier sign is present in this condition
Smooth muscle hamartoma
Which immunochemical stain/s is/are strongly and diffusely expressed in virtually all piloleiomyomas:
A. H-Caldesmon
B. Desmin
C. Muscle-specific actin
D. B and C
E. All of the above
E
Delineation of MPNST from a benign variant includes the following except:
A. Marked cell crowding
B. Generalized nuclear enlargement (5 times normal)
C. Nuclear hyperchromasia
D. Mitotic rate greater than 5 per 10HPF
E. None of the above
B. Generalized nuclear enlargement (5 times normal)
The partial or even complete loss of ___ expression in malignant peripheral nerve sheath tumor is one of the most useful distinctions from benign tumors:
S100
Risk factors for metastatic spread of leiomyosarcoma
A. Tumor size
B. Degree of subcutaneous involvement
C. High histologic grade
D. B and C
E. All of the above
E
