Tumors

Question 1

Extraskeletal manifestations of Jaffe-Campanacci?

Answer 1

café-au-lait spots, mental retardation, hypogonadism/cryptorchidism, ocular abnormalities, cardiovascular malformations

Question 2

LICHTENSTEIN’S RULE?

Answer 2

any malignant-looking lesion in the tibia or femur that is not metastasis and does not show characteristics of another primary, the primary consideration should be fibrosarcoma

Question 3

Age range for MESENCHYMAL CHONDROSARCOMA?

Answer 3

2nd-4th decades (average 25 yoa, note- younger than conventional)

Question 4

What are giant cells?

Answer 4

multinucleated macrophages

Question 5

GCT more commonly malignant in whom?

Answer 5

Males, 3:1

Question 6

What is Goltz Syndrome? What about Gorlin-Goltz?

Answer 6

focal dermal hypoplasia with ocular defects and multifocal GCT’s
GG is a phakomatosis = basal cell nevus syndrome

Question 7

EXTRAGNATHIC GIANT CELL REPARATIVE GRANULOMA OF BONE may also be referred to as what?

Answer 7

Solid variant of an ABC

Question 8

MFH may arise secondary to what other diseases of bone?

Answer 8

infarction, intraosseous lipoma, Paget’s disease, post-radiation therapy

Question 9

Mets from where might simulate liposarcoma?

Answer 9

Renal cell carcinoma

Question 10

Klippel-Trénaunay-Weber vs. Osler-Weber-Rendu syndrome

Answer 10

Rendu - AVMs, aka Hereditary haemorrhagic telangiectasia

Klippel - capillary malformations, soft-tissue or bone hypertrophy, and varicose veins or venous malformations. It is considered an angio-osteo-hypertrophic syndrome.

Question 11

M/C tumor to cause hypophosphatemic osteomalacia?

Answer 11

HEMANGIOPERICYTOMA

Question 12

cookie bite lesion most commonly seen with what primary?

Answer 12

bronchogenic ca

Question 13

How often is Ewing’s sclerotic?

Answer 13

1/3 of the time

Question 14

Specific histo finding for Ewings?

Answer 14

70-100% PAS +ve for glycogen (test for glycogen granules in cytoplasm of tumor cells)

Question 15

Lab finding for PNET?

Answer 15

produces neuron-specific enolase

Question 16

M/C malignant disease of kids in tropical Africa

Answer 16

BURKITT’S LYMPHOMA

Question 17

what is MYCOSIS FUNGOIDES?

Answer 17

Form of malignant T-cell lymphoma, 4th-5th decade, M/C male, initially presents as a nonspecific skin ulceration

Question 18

eponym for mult. myeloma?

Answer 18

Kahler’s disease

Question 19

“Pad sign” in MM?

Answer 19

Amyloid deposition next to joint

Question 20

POEMS syndrome?

Answer 20

POEMS syndrome : Polyneuropathy, Organomegaly (hepatosplenomegaly, impotence, gynecomastia, amenorrhea), Endocrinopathy (edema, perspiration, hirsituism), M proteins, Skin changes (thickened, pigmented)
Lesions are not infrequently sclerotic in this form of MM

Question 21

chloroma?

Answer 21

associated with leukemia (AML), aka GRANULOCYTIC SARCOMA

Question 22

MC location for intraosseous epidermoid cyst?

Answer 22

In the hands, the left side and the middle finger represent preferred sites of involvement.

Question 23

Triad in Hans-Schuller-Christian disease?

Answer 23

10% have a triad of uni/bilateral exopthalmos, diabetes insipidus, single/multiple lytic lesions