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Clin Med 1 - Exam 5 > Porphyrias and Sarcomas > Flashcards

Porphyrias and Sarcomas Flashcards

1
Q

Symptoms of acute intermittent porphyria

A

Abdominal pain most common

Neuro and visceral symptoms

No cutaneous manifestations

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2
Q

These sarcomas are described as indolent (slow progressing, low metastasis potential)

A
  • Chondrosarcoma
  • Kaposi sarcoma
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3
Q

How to test porphyrin levels?

A

Plasma or urine samples

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4
Q

Environmental exposures that can increase risk for these three sarcomas:
- Osteosarcoma
- Mesothelioma
- Angiosarcoma

A
  • Radiation (osteosarcoma)
  • Asbestos (mesothelioma)
  • PVC, arsenic (angiosarcoma)
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5
Q

Which sarcoma is mostly seen in older adults?

A

Chondrosarcoma

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6
Q

Variegate porphyria symptoms

A

Neurovisceral symptoms and photosensitivity

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7
Q

Ewing sarcoma symptoms

A

Pain in bones that continues through night/doesn’t get better with rest

Systemic symptoms (fever, malaise)

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8
Q

These two diseases are risk factors for osteosarcoma

A

Retinoblastoma and Paget’s disease

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9
Q

Inheritance pattern of acute intermittent porphyria

A

Autosomal dominant

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10
Q

Osteosarcoma appearance on xray

A
  • Sunburst appearance
  • Cancer in bone and surrounding tissue
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11
Q

Diagnosis imaging for Ewing sarcoma

A

Xray
- poorly marginated destructive lesion with a moth-eaten or onion skin appearance

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12
Q

Sarcomas are cancers of the …

A

“Flesh” = Bones and soft tissues

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13
Q

Patients with this porphyria have an increased risk of hepatocellular carcinoma

A

Porphyria cutanea tarda

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14
Q

Most common porphyria

A

Porphyria cutanea tarda

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15
Q

This sarcoma is associated with HHV-8

A

Kaposi sarcoma

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16
Q

Most common sarcoma of bones

A

Osteosarcoma

17
Q

Clinical manifestations of sarcomas of soft tissue

A

Painless mass on extremity or trunk without history of injury

18
Q

Kaposi sarcoma is most common in these patients

A

Immunosuppressed (HIV/AIDS)

19
Q

Are sarcomas more common in young or old people?

A

More common in kids

20
Q

Congenital erythropoietic porphyria treatments

A
  • Avoid sunlight, use sunscreen
  • Beta-carotene
  • RBC transfusions
  • Bone marrow transplant
21
Q

Average age for patients to present with Ewing sarcoma

A

10-20 years old

22
Q

Porphyria cutanea tarda is associated with this metabolic disorder

A

Hemochromatosis

23
Q

Which porphyria is not inherited?

A

Porphyria cutanea tarda

24
Q

Porphyria cutanea tarda symptoms

A

Mostly a blistering skin condition
- vesicles on sun exposed area of the skin

25
Q

Treatments for porphyria cutanea tarda

A
  • Abstinence from alcohol - already flooded with iron
  • Phlebotomy to remove iron from blood
26
Q

Are systemic symptoms more common in Ewing sarcoma or osteosarcoma?

A

Ewing sarcoma

  • systemic symptoms generally absent in osteosarcoma
27
Q

Symptoms of congenital erythropoietic porphyria

A
  • Pink to dark-brown staining of diapers
  • Photosensitivity
  • Erythrodontia - red fluorescent teeth under UV light
28
Q

Ewing sarcoma most commonly shows up in these bones

A
  • Long bones of the extremities (femur, humerus, tibia)
  • Flat bones of the pelvis (ileum)
29
Q

Risk of osteosarcoma in higher in children with a history of this other cancer

A

Retinoblastoma (no red reflex)

30
Q

Inheritance pattern of congenital erythropoietic porphyria

A

Autosomal recessive

31
Q

Treatment for variegate porphyria

A

Protection from sunlight

32
Q

Kaposi sarcoma symptoms

A

Dark purple lesions on lower legs/feet initially (eventually in oral mucosa)

33
Q

Multiple sarcomas can run in a family who pass on this gene mutation to their children

A

P53 mutation (oncogene)

34
Q

Average age of chondrosarcoma patients

A

> 75 years old

35
Q

Things that trigger attacks of acute intermittent porphyria

A
  • Drugs (oral contraceptives)
  • Endocrine factors
  • Reduced caloric intake
36
Q

Treatments for acute intermittent porphyria

A

IV dextrose and hematin

(to slow/suppress heme synthesis)

37
Q

Describe the cause of porphyrias

A

Defect in enzyme responsible for heme synthesis

Results in toxic accumulation of heme precursors

38
Q

Appearance of chondrosarcoma on x-ray

A
  • Lytic appearance
  • Intralesional calcifications
39
Q

Which porphyria has no skin related symptoms?

A

Acute intermittent porphyria

Clin Med 1 - Exam 5 (13 decks)

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