Updated: 10/17/2018

Vascular Tumors

Topic
Review Topic
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Questions
4
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Evidence
7
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Snapshot
  • A 34-year-old man presents with a rash. He states he recently started feeling fatigued and a purple rash appeared a few weeks ago. On physical exam, there are dozens of purple papules and nodules on the skin and on the oral mucosa. A biopsy reveals lymphocytic infiltration. Additional viral testing is sent for further workup. (Kaposi sarcoma)
Introduction
  • Benign or malignant vascular proliferation
    • most commonly, these neoplasms manifest cutaneously
Benign Vascular Neoplasms
  • Strawberry hemangioma
    • clinical definition
      • benign capillary hemangioma of infancy
        • also known as infantile hemangioma
    • regresses spontaneously within a few years
    • treatment is typically not necessarily
  • Cherry angioma
    • clinical definition
      • benign capillary hemangioma of adulthood
      • more frequent in elderly patients
    • does not regress spontaneously
    • treatment is typically not necessarily
  • Cavernous hemangioma
    • clinical definition
      • benign tumor of liver and spleen
        • risk of rupture with increasing size
    • associated conditions
      • von-Hippel-Lindau syndrome
    • treatment is typically not necessarily
  • Pyogenic granuloma
    • clinical definition
      • polypoid lobulated capillary hemangioma of the skin and mucosa
        • easily ulcerates and bleeds
        • often occurs on the gingiva
    • associated conditions
      • trauma
      • pregnancy
    • treatment
      • the lesion can be surgically removed if it causes excessive discomfort
Malignant Vascular Neoplasms
  • Kaposi sarcoma
    • clinical definition
      • endothelial malignancy that most commonly affects the skin, characterized by purple papules and nodules
      • can also affect the mouth, gut, and lungs
      • characterized by a lymphocytic infiltrate
    • associated conditions
      • human herpesvirus-8 infection
      • human immunodeficiency virus infection
    • treatment
      • antiretroviral therapy 
      • if refractory
        • adriamycin
        • vinblastine
  • Angiosarcoma
    • clinical definition
      • malignant vascular neoplasm affecting the head, neck, chest, and liver
      • more common in sun-exposed areas in the elderly population
      • very aggressive malignancy
    • associated conditions
      • radiation therapy
      • hepatic angiosarcoma is specifically associated with a "VAT of chemicals"
        • Vinyl chloride
        • Arsenic
        • Thorotrast
      • lymphangiosarcoma is associated with
        • chronic post-mastectomy lymphedema
    • treatment
      • surgical excision
Other Neoplasms
  • Glomus tumor
    • clinical definition
      • painful benign red-blue tumor of the glomus body
        • glomus body is a thermoregulatory agent, which can shunt blood towards or away from the skin surface
      • found under the fingernails
    • treatment
      • surgical excision
  • Cystic hygroma
    • clinical definition
      • cavernous lymphangioma of the neck
        • “webbed neck”
    • associated conditions
      • Turner syndrome
  • Bacillary angiomatosis 
    • clinical definition
      • benign capillary skin lesions caused by Bartonella henselae infections
      • characterized by neutrophilic infiltrate
    • associated conditions
      • acquired immunodeficiency disease syndrome (AIDS)
      • cat-scratch disease
    • treatment
      • antibiotic therapy
 

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Questions (4)
Lab Values
Blood, Plasma, Serum Reference Range
ALT 8-20 U/L
Amylase, serum 25-125 U/L
AST 8-20 U/L
Bilirubin, serum (adult) Total // Direct 0.1-1.0 mg/dL // 0.0-0.3 mg/dL
Calcium, serum (Ca2+) 8.4-10.2 mg/dL
Cholesterol, serum Rec: < 200 mg/dL
Cortisol, serum 0800 h: 5-23 μg/dL //1600 h:
3-15 μg/dL
2000 h: ≤ 50% of 0800 h
Creatine kinase, serum Male: 25-90 U/L
Female: 10-70 U/L
Creatinine, serum 0.6-1.2 mg/dL
Electrolytes, serum  
Sodium (Na+) 136-145 mEq/L
Chloride (Cl-) 95-105 mEq/L
Potassium (K+) 3.5-5.0 mEq/L
Bicarbonate (HCO3-) 22-28 mEq/L
Magnesium (Mg2+) 1.5-2.0 mEq/L
Estriol, total, serum (in pregnancy)  
24-28 wks // 32-36 wks 30-170 ng/mL // 60-280 ng/mL
28-32 wk // 36-40 wks 40-220 ng/mL // 80-350 ng/mL
Ferritin, serum Male: 15-200 ng/mL
Female: 12-150 ng/mL
Follicle-stimulating hormone, serum/plasma Male: 4-25 mIU/mL
Female: premenopause: 4-30 mIU/mL
midcycle peak: 10-90 mIU/mL
postmenopause: 40-250
pH 7.35-7.45
PCO2 33-45 mmHg
PO2 75-105 mmHg
Glucose, serum Fasting: 70-110 mg/dL
2-h postprandial:<120 mg/dL
Growth hormone - arginine stimulation Fasting: <5 ng/mL
Provocative stimuli: > 7ng/mL
Immunoglobulins, serum  
IgA 76-390 mg/dL
IgE 0-380 IU/mL
IgG 650-1500 mg/dL
IgM 40-345 mg/dL
Iron 50-170 μg/dL
Lactate dehydrogenase, serum 45-90 U/L
Luteinizing hormone, serum/plasma Male: 6-23 mIU/mL
Female: follicular phase: 5-30 mIU/mL
midcycle: 75-150 mIU/mL
postmenopause 30-200 mIU/mL
Osmolality, serum 275-295 mOsmol/kd H2O
Parathyroid hormone, serume, N-terminal 230-630 pg/mL
Phosphatase (alkaline), serum (p-NPP at 30° C) 20-70 U/L
Phosphorus (inorganic), serum 3.0-4.5 mg/dL
Prolactin, serum (hPRL) < 20 ng/mL
Proteins, serum  
Total (recumbent) 6.0-7.8 g/dL
Albumin 3.5-5.5 g/dL
Globulin 2.3-3.5 g/dL
Thyroid-stimulating hormone, serum or plasma .5-5.0 μU/mL
Thyroidal iodine (123I) uptake 8%-30% of administered dose/24h
Thyroxine (T4), serum 5-12 μg/dL
Triglycerides, serum 35-160 mg/dL
Triiodothyronine (T3), serum (RIA) 115-190 ng/dL
Triiodothyronine (T3) resin uptake 25%-35%
Urea nitrogen, serum 7-18 mg/dL
Uric acid, serum 3.0-8.2 mg/dL
Hematologic Reference Range
Bleeding time 2-7 minutes
Erythrocyte count Male: 4.3-5.9 million/mm3
Female: 3.5-5.5 million mm3
Erythrocyte sedimentation rate (Westergren) Male: 0-15 mm/h
Female: 0-20 mm/h
Hematocrit Male: 41%-53%
Female: 36%-46%
Hemoglobin A1c ≤ 6 %
Hemoglobin, blood Male: 13.5-17.5 g/dL
Female: 12.0-16.0 g/dL
Hemoglobin, plasma 1-4 mg/dL
Leukocyte count and differential  
Leukocyte count 4,500-11,000/mm3
Segmented neutrophils 54%-62%
Bands 3%-5%
Eosinophils 1%-3%
Basophils 0%-0.75%
Lymphocytes 25%-33%
Monocytes 3%-7%
Mean corpuscular hemoglobin 25.4-34.6 pg/cell
Mean corpuscular hemoglobin concentration 31%-36% Hb/cell
Mean corpuscular volume 80-100 μm3
Partial thromboplastin time (activated) 25-40 seconds
Platelet count 150,000-400,000/mm3
Prothrombin time 11-15 seconds
Reticulocyte count 0.5%-1.5% of red cells
Thrombin time < 2 seconds deviation from control
Volume  
Plasma Male: 25-43 mL/kg
Female: 28-45 mL/kg
Red cell Male: 20-36 mL/kg
Female: 19-31 mL/kg
Cerebrospinal Fluid Reference Range
Cell count 0-5/mm3
Chloride 118-132 mEq/L
Gamma globulin 3%-12% total proteins
Glucose 40-70 mg/dL
Pressure 70-180 mm H2O
Proteins, total < 40 mg/dL
Sweat Reference Range
Chloride 0-35 mmol/L
Urine  
Calcium 100-300 mg/24 h
Chloride Varies with intake
Creatinine clearance Male: 97-137 mL/min
Female: 88-128 mL/min
Estriol, total (in pregnancy)  
30 wks 6-18 mg/24 h
35 wks 9-28 mg/24 h
40 wks 13-42 mg/24 h
17-Hydroxycorticosteroids Male: 3.0-10.0 mg/24 h
Female: 2.0-8.0 mg/24 h
17-Ketosteroids, total Male: 8-20 mg/24 h
Female: 6-15 mg/24 h
Osmolality 50-1400 mOsmol/kg H2O
Oxalate 8-40 μg/mL
Potassium Varies with diet
Proteins, total < 150 mg/24 h
Sodium Varies with diet
Uric acid Varies with diet
Body Mass Index (BMI) Adult: 19-25 kg/m2
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(M1.CV.91) A 68-year-old female presents after noting the onset 3 months ago of an area of erythema on the anterior aspect of her left upper arm. Over the next several months, this lesion progressed to include numerous purple papules with areas of apparent necrosis as well as an enlarging underlying mass. Figure A depicts the patient's arm at her current presentation. She reports that her left arm has felt "heavy" and appeared swollen in the past. Which of the following elements of this patient's history would have most likely contributed to the development of her current condition? Review Topic

QID: 100607
FIGURES:
1

Multiple recurrent staphylococcal skin abscesses treated with IV antibiotics and serial I&Ds

33%

(4/12)

2

Progressively debilitating polyarticular osteoarthritis

0%

(0/12)

3

Modified radical mastectomy with subsequent radiation therapy 12 years ago

58%

(7/12)

4

Remote anterior shoulder subluxation after an accidental injury 30 years ago

0%

(0/12)

5

Former 1 pack-per-day smoker, who quit 10 years ago

8%

(1/12)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 3

(M1.CV.52) A 45-year-old woman presents to your clinic concerned about changes in her skin over the past few years as shown in Figure A. The lesions do not hurt or bleed and she is not bothered by their appearance but she is somewhat concerned that the lesions may be a sign of a more serious underlying medical condition. She is interested in reviewing a treatment plan that will prevent the lesions from causing adverse long-term effects. What treatment is required for this patient's skin lesions? Review Topic

QID: 104263
FIGURES:
1

Immediate removal

1%

(1/87)

2

Biopsy

9%

(8/87)

3

Topical corticosteroids

2%

(2/87)

4

Regular follow-up to monitor regression of lesions

21%

(18/87)

5

No treatment required

66%

(57/87)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 5

(M1.CV.67) A 51-year-old woman presents to the dermatologist with concern for a new skin lesion (Image A). You note two similar lesions on her back. Which of the following is a true statement about these lesions? Review Topic

QID: 100583
FIGURES:
1

They will likely grow rapidly.

11%

(1/9)

2

They will likely regress spontaneously.

0%

(0/9)

3

They may be associated with von Hippel-Lindau disease.

0%

(0/9)

4

They will likely increase in number over time.

67%

(6/9)

5

They must be followed closely for concern of malignancy.

11%

(1/9)

M1

Select Answer to see Preferred Response

PREFERRED RESPONSE 4
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