| Introduction |
 Benign vascular neoplasm in soft tissue
- may be cutaneous, subcutaneous, or intramuscular
- capillary (superficial) or cavernous (deep)
- Epidemiology
- incidence
- males and females incidence is equal
- <1% of all benign vascular tumors
- demographics
- more common in patients <30 years of age
- location
- intramuscular is commonly found deep in the lower extremities
- most commonly found as isolated lesions, except for diffuse hemangioma, a rare childhood form which extensively involves a single limb
- common in the hand
- Pathophysiology
- caused by an error in the new formation of a segment of the vascular system
- hormones may play role in modulation
- 20% are associated with trauma
- Prognosis
- infantile hemangiomas involute by age 7
- no incidence of malignant transformation
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| Presentation |
- Symptoms
- pain of variable intensity depending on activity level
- symptoms of vascular engorgement if hemangioma is large
- aching
- heaviness
- swelling
- Physical exam
- examine patient in both supine and standing position
- lower extremity lesions will fill-up after several minutes of standing
- inspection
- mass of variable size depending on activity level
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| Imaging |
- Radiograph
- may show small phleboliths (calcifications) inside the lesion
- erosion into adjacent bone
- MRI with gadolinium
- differentiates these benign lesions from arteriovenous malformations and angiosarcomas
- increased signal on T1- and T2- weighed images
- focal areas of low-signal are a sign of blood flow or calcifications
- heterogeneous lesion with numerous small blood vessels and fatty infiltration (bag of worms)
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| Studies |
- Histology
- gross
- varies depending on whether it is capillary type or cavernous type
- color spectrum varies from red to tan to yellow
- micro
- no malignant cells noted by cellular pleomorphism noted
- many vascular dilations with large nuclei filled with erythrocytes
- vascular lumens infiltrated with muscle fibers (intramuscular type)
- cavernous shows large vessels with lots of fatty tissue
|
| Differential |
- Other vascular malformations
- arteriovenous malformations
- cavernous hemangiomas
- angiomatosis
- vascular ectasia
- Sarcoma
- important to distinguish from sarcoma
|
| Treatment |
- Nonoperative
- observation, NSAIDS, vascular stockings, and activity modification
- indications
- first line of treatment
- childhood lesions
- sclerotherapy or embolization
- performed by interventional radiology (IR)
- indications
- large, painful lesions that fail NSAID and vascular stockings therapy
- Operative
- marginal excision
- indications
- small lesions of the hand
- wide surgical resection
- indications
- lesions resistant to nonoperative management
- outcomes
- high incidence of local recurrence
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| Complications |
- Kasabach-Merritt syndrome
- rare complications caused by entrapped platelets leading to a possbly fatal coagulopathy
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Please Rate Educational Value!
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3.0
t-8069
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Average 3.0 of 6 Ratings
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Qbank (2 Questions)
TAG
(OBQ06.119)
A 17-year-old male presents to your office with hand swelling and intermittent pain. His pain occurs when his hand is held in a dependent position or when he is swimming. While he was born with a birthmark on his hand, he has noticed it is progressively getting bigger. Clinical photograph, radiographs, and histology are shown. What is the most likely diagnosis?
Review Topic
DISCUSSION:
The clinical history, photograph, radiographs, and histology are all consistent with a benign hemangioma. Hemangiomas are very common benign tumors which can become painful when engorged with blood secondary to dependent positions or physical activity. The clinical photograph shows the associated skin changes which occur when hemangiomas are located in subcutaneous locations. The radiographs show classic phleboliths (calcifications within blood vessels) on the ulnar side of the radiograph. The histology photograph shows no cellular atypia or malignant features. Rather, multiple large endothelial-lined vascular dilations are seen each filled with erythrocytes.
REFERENCES:
1.
Menendez LR 9ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopedic Surgeons, 2002, pp243-253
2.
Weiss SW, Goldblum JR: Benign tumors and tumor-like lesions of blood vessels, in Enxinger FM, Weiss SW 9eds): Soft Tissue Tumors. St. Louis, MO, Mosby, 2001, pp837-890.
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Please Rate Educational Value!
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3.0
q-305
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Average 3.0 of 8 Ratings
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TAG
(OBQ04.13)
A 30-year-old female presents with a painful posterior knee mass. The mass gets larger and more painful with activity. Examination reveals a boggy soft tissue mass about her knee. Radiograph and MRI are shown in Figures A and B. What is the most likely diagnosis?
Review Topic
DISCUSSION:
This patients history, physical exam, and imaging studies are suggestive of a hemangioma. Pain in hemangiomas is thought to occur due to vascular engorgement secondary to activity and increased blood flow to the lesion. The calcification on the plain radiograph and the fat of the T1 MRI are diagnostic for hemangioma. Current treatment for symptomatic hemangiomas includes some combination of sclerotherapy or vascular coiling, with surgical excision reserved for few cases. The two attached reviews by Gilbert et al and Damron et al review the history, diagnosis, treatment, and controversial issues in soft tissue sarcomas and benign sarcoma like conditions such as hemangiomas. Current treatment for soft tissue sarcomas includes radiotherapy and surgical excision. While many centers in the world use chemotherapy for soft tissue sarcomas, the data supporting its use is quite limited and likely too controversial to be tested. With regard to the other answer choices, synovial sarcoma and lipoma can show calcification on radiographs, but they are usually not painful and image differently on T1 MRI. Parosteal osteosarcoma typically occurs in this location (posterior distal femur) but occurs as a lesion stuck on the bone. PVNS can have a similar appearance on imaging, but doesn't cause this type of pain.
REFERENCES:
1.
Gilbert NF, Cannon CP, Lin PP, Lewis VO. Soft-tissue sarcoma. J Am Acad Orthop Surg. 2009 Jan;17(1):40-7. Review.
PMID:19136426 (Link to Abstract)
2.
Damron TA, Beauchamp CP, Rougraff BT, Ward WG Sr. Soft-tissue lumps and bumps. Instr Course Lect. 2004;53:625-37. Review.
PMID:15116652 (Link to Abstract)
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Please Rate Educational Value!
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2.0
q-124
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Average 2.0 of 8 Ratings
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Level of Evidence 5 and Other Journal Articles (includes Case Reports, Expert Opinions,
Personal Observations, and Biomechanic Studies)
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Damron TA, Beauchamp CP, Rougraff BT, Ward WG Sr. Soft-tissue lumps and bumps. Instr Course Lect. 2004;53:625-37. Review.
PMID:15116652 (Link to Abstract)
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Gilbert NF, Cannon CP, Lin PP, Lewis VO. Soft-tissue sarcoma. J Am Acad Orthop Surg. 2009 Jan;17(1):40-7. Review.
PMID:19136426 (Link to Abstract)
Textbooks
- Review of Orthopaedics, 6th Edition, Mark D. Miller MD, Stephen R. Thompson MBBS MEd FRCSC, Jennifer Hart MPAS PA-C ATC, an imprint of Elsevier, Philadelphia, Copyright 2012
- AAOS Comprehensive Orthopaedic Review, Jay R. Leiberman. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2009
- Orthopaedic Knowledge Update 10, John M Flyn. Published by American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2011
- Hoppenfeld SP. Surgical Exposures in Orthopaedics: The Anatomic Approach. Lipponcott, Williams, and Wilkins, Philadelphia, PA, Copyright 2009
- Orthopaedic In-training Examination (OITE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
- Self-Assessment Examination (SAE) Questions 2004-2012, American Academy of Orthopaedic Surgeons, Rosemont IL. Copyright 2004-2012
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Menendez LR 9ed): Orthopaedic Knowledge Update: Musculoskeletal Tumors. Rosemont, IL, American Academy of Orthopedic Surgeons, 2002, pp243-253
-
Weiss SW, Goldblum JR: Benign tumors and tumor-like lesions of blood vessels, in Enxinger FM, Weiss SW 9eds): Soft Tissue Tumors. St. Louis, MO, Mosby, 2001, pp837-890.
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