Arch Hand Microsurg.  2020 Sep;25(3):201-206. 10.12790/ahm.20.0028.

Multiple Huge Tendinous Xanthomas with Normal Lipid Profiles in All Extremities

• Seo DH
• Roh SG
• Shin JY
• An MY
• Kim JL
• Chang SC
• Lee NH

• KMID: 2505901
• DOI: http://doi.org/10.12790/ahm.20.0028

Abstract

Xanthomas are grayish-yellow masses composed of lipid-filled foamy histiocytes and are usually accompanied by familial hypercholesterolemia or some other disease associated with dysfunctional lipid metabolism. Here, we report a case of multiple huge tendinous xanthomas with normal lipid profiles involving all extremities. These masses were large enough to cause pain, dysfunction of extremities, and cosmetic compromise and the condition was accompanied by cerebrotendinous xanthomatosis. Due to the presence of many masses in all extremities, a two-stage operation was planned with a time gap of several months. At 1-month follow-up visits after first and second surgeries, although extension of the left middle finger was poor as a result of sacrificing the 3rd extensor digitorum communis tendon, no problems such as wound dehiscence, hematoma formation, or infection at operative sites were noted.

Keyword

Cerebrotendinous xanthomatosis; Tendinous xanthoma; Hypercholesterolemia

Figure

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  Fig. 1. The large, firm nontender masses with ulceration were on tendons of left hand, right elbow, both ankles and feet.

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  Fig. 2. In T1-weighted image, xanthoma infiltrated into the tendon of flexor carpi ulnaris was seen in left wrist. There was no definitive involvement of ulnar nerve and artery (arrowhead). The 2nd to 4th extensor tendons were involved by the xanthoma on the left dorsum of hand (arrow).

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  Fig. 3. Histopathology finding showing lipid accumulation (arrow), foam cell (arrowhead), histiocytes, and lymphocytes (H&E, ×400).

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  Fig. 4. During subtotal excision for mass on the left wrist, ulnar nerve and artery was not involved and preserved (A). (B, C) For the large masses were on both Achilles tendon, subtotal excision was done preserving Achilles tendon.

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  Fig. 5. The masses involved in 3rd extensor digitorum communis tendon (A) and both tendons of extensor hallucis longus (B) was completely excised and then tendons were sacrificed due to severe infiltration into the tendons.

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  Fig. 6. Extension of the left middle finger was poor as a result of sacrificing the 3rd extensor digitorum communis tendon after surgery.

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