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Pathology II > Diseases of Bone- Giant Cell Lesions and LCH > Flashcards

Diseases of Bone- Giant Cell Lesions and LCH Flashcards

1
Q

describe cherubism

A
  • autosomal dominant
  • bilateral jaw lucencies with giant cell histology
  • variable clinical course
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2
Q

what is the clinical presentation for cherubism

A

maxillary expansion

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3
Q

what is the radiographic appearance of cherubism

A

multiple multilocular RL

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4
Q

what is the resolution with cherubism

A
  • can undergo spontaneous resolution
  • no need for surgery unless cosmetic
  • RL will resolve and clinical expansion will resolve
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5
Q

what is the histo for cherubism

A
  • similar to central giant cell tumor
  • variable numbers of multinucleated giant cells
  • extravasated RBCs
  • eosinophilic, cuff like deposits surrounding small blood vessels
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6
Q

describe central giant cell granuloma, predilection

A
  • youn individuals 10-30 years
  • females
  • unilocular or multilocular lucency
  • anterior jaw lesion, often crosses midline
  • cortical expansion, perforation uncommon, resorption of tooth roots
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7
Q

what is the radiographic presentation for central giant cell granuloma

A
  • multilocular or unilocular RL
  • can cause root resorption
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8
Q

what is the tx for central giant cell granuloma

A
  • curettage - 15-20% recurrencee
  • intralesional corticosteroids
  • calcitonin
  • interferon alpha2a
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9
Q

what is the histo presentation of central giant cell granuloma

A
  • cellular tumor stroma composed of benign, mononuclear cells
  • multinucleated giant cells: osteoclasts
  • extravasated RBCs
  • hemosiderin: brownish
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10
Q

what is the histologic differential dX for central giant cell granuloma

A
  • central giant cell lesion
  • cherubism
  • hyperparathyroidism (brown tumor)
  • giant cell tumor
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11
Q

describe the giant cell tumor of long bone

A
  • true neoplasm
  • epiphyses of long bones of older adults
  • metastasis of histologically benign tumor of lung
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12
Q

describe langerhans cell histiocytosis

A

neoplastic proliferation of langerhand histiocytes- clonal
- wide spectrum of involvement and biologic behavior- from solitary bone lesions to generalized bone lesions with organ and soft tissue lesions

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13
Q

what are the three categories for langerhans cell histiocytosis for tx and prognosis and describe each

A
  • chronic focal: solitary lesion in one bone, no soft tissue or organ involvement (eosinophilic granuloma)
  • chronic disseminated- mulitple bones, organs, lymph nodes and skin (hand- schuller- christian disease)
  • acute disseminated- involves most organs, lymph nodes, bone marrow and skin of infants ( letterer-Siwe disease)
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14
Q

what is the most common form of langerhans cell histiocytosis

A

chronic focal

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15
Q

describe the radiographic appearance and predilection for chronic focal langerhans cell histiocytosis

A
  • teens and young adults
  • area of discomfort
  • punched out lucency
  • periapical pathoses
  • advanced perio disease- teeth floating in air
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16
Q

describe chronic disseminated langerhans cells histiocytosis and who it affects

A
  • hand- schuller christian disease
  • most common in children under 10 years
  • bone lesions similar to chronic focal form and soft tissue lesions
17
Q

describe acute disseminated langerhans cell histiocytosis and who it affects

A
  • letterer Siwe disease
  • infants
  • rapidly fatal course
  • disseminated involvement of organs, lymph nodes, bone marrow and skin by anaplastic cells
18
Q

what is the ddx for langerhans cell histiocytosis and how to tell difference

A
  • mulitple myeloma
  • multiple myeloma occurs in older pt
19
Q

what is the histopath for langerhans cell histiocytosis

A
  • sheets of large histiocytic cells with eosinophilic cytoplasma and centrally placed nuclei
  • focal abundant infiltrates of eosinophils
  • birbeck granules
20
Q

what is the staining used for langerhans cell histiocytosis

A

S100 - if positive it stains brown

21
Q

what is the tx for langerhans cell histiocytosis

A
  • focal chronic form- curettage
  • diffuse forms- chemotheraoy
  • long term folllow up for recurrence and development of new lesions
22
Q
A

Pathology II (19 decks)

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