Benign Vascular and Malignant Vascular Tumors

Earl W. Brien and Stuart H. Kuschner

History and Clinical Presentation

Case A. A 35-year-old right hand dominant woman complains of a painful mass about the tip of the index finger of 6 months' duration. She states that the pain is aggravated by cold and often radiates proximally after tactile stimulation. She denies any history of trauma, puncture wounds, or fever.

Case B. A 55-year-old right hand dominant woman with a history of swelling about the left upper extremity after radical mastectomy and lymph node dissection 10 years prior complains of a painless, enlarging mass in the left hand of 4 months' duration. She denies any history of trauma, puncture wounds, or fever.

Physical Examination

Case A. The patient has 1.5-cm, firm, nonmobile mass about the tuft of the index finger. The lesion revealed a blue-red hue subungual. She has lancinating pain proximally upon pinprick about the digit. She has no proximal adenopathy and the remainder of her examination is within normal limits.

Case B. The patient has 2.5-cm dorsoradial wrist mass superimposed on a nonpitting, brawny edema. It is firm, superficial, nontender, and nonmobile, without any warmth or erythema. The lesion did not transilluminate and there was a purple hue about the skin. She has full range of motion of the wrist and hand and is neurovas-cularly intact to all digits. She has no proximal adenopathy and the remainder of her examination is within normal limits.

Diagnostic Studies

Case A. Anteroposterior, lateral, and oblique radiographs of the hand show a soft tissue mass about the tuft with a well circumscribed scalloping about the distal phalanx (Fig. 87-1).

Figure 87—1. Well-circumscribed lesion, distal phalanx.

Figure 87—1. Well-circumscribed lesion, distal phalanx.

Figure 87—2. Magnetic resonance imaging through the base of the hand of a dorso-radial mass.

Case B. Anteroposterior, lateral, and oblique radiographs of the hand show a soft tissue mass dorsally without calcification. Magnetic resonance imaging (MRI) (Fig. 87—2) reveals a 2.5-cm mass, intermediate signal onTl- and bright signal on T2-weighted imaging. Laboratory studies were unremarkable.

Differential Diagnoses

Case A

Glomus tumor Melanoma

Inclusion cyst/foreign body granuloma Infection

Metastatic squamous carcinoma of the lung Case B

Ganglion cyst

Giant cell tumor of the tendon sheath Soft tissue sarcoma Other benign tumors Atypical infection/synovitis Inclusion cyst/foreign body granuloma

Pathologic Examination

Case A. Histopathologic examination revealed small round blue cells forming vascular structures in a myxoid stroma (Fig. 87-3). On high power (Fig. 87-4), central clearing or punched out nuclei are seen surrounded by a faintly staining cytoplasm best seen with periodic acid-Schiff (PAS) stain or toluidine blue stain. Ultrastructural findings reveal myofilaments closely related to smooth muscle.

Case B. Histopathologic examination (Fig. 87-5) revealed communicating vascular channels dissecting between the dermal collagen and fat cells. Cells lining the lumina reveal hyperchromatic nuclei and mild to moderate atypia with occasional shedding of cells into the lumen. Immunohistochemical stains revealed vimentin and factor VIII antigen positivity.

Pictures Muscle Tumors Found Mri

Figure 87—2. Magnetic resonance imaging through the base of the hand of a dorso-radial mass.

Glomus Cells
Figure 87—3. Glomus tumor, low-power magnification.
Fatty Tumors The Hand

Figure 87—4. Glomus tumor, high-power magnification.

Sucquet Hoyer Glomus Cell

Figure 87—5. Vascular channels, with adjacent fat cells and collagen.

Diagnosis

Case A. Glomus tumor.

The glomus tumor resembles the modified smooth muscle cells of the glomus body, which is a specialized form of arteriovenous anastomosis that regulates temperature and is most commonly found subungual. A series of specialized arterioles blend into a thick walled lumen known as the Sucquet-Hoyer canal, which is lined by cuboidal endothelial cells, surrounded by smooth muscles and scattered rounded, glomus cells.

Glomus tumors are uncommon, usually diagnosed in adulthood and have a predilection for women in the subungual location. The lesion often develops as a red-blue nodule in the deep dermis and causes radiating pain often elicited by cold or tactile stimulation. Radiographically, a soft tissue mass about the pulp, with or without a smooth bordered erosion of the distal phalanx, may be seen. Differential diagnosis includes inclusion cyst, foreign body granuloma, infection, and, less likely, metasta-tic disease from squamous carcinoma of the lung. Histologically, a small, often well-circumscribed vascular lesion surrounded by round, blue glomus cells is seen in a myxoid or collagenous stroma. On high power, the glomus cells seen are characterized by their punched-out rounded nucleus with amphophilic cytoplasm forming a chicken wire background. These are best seen using PAS stain. Histologic differential diagnosis includes glomangiomyoma and glomangiosarcoma.

Glomus tumors are benign and are best treated with local excision. Recurrence may be seen in inadequate excision and occur in ~10% of cases.

Case B. Angiosarcoma.

Angiosarcoma associated with lymphedema (Stewart-Treves disease).

The overwhelming majority of soft tissue tumors about the hand and wrist are benign. Ganglion cyst accounts for over 70% of lesions about the wrist; however, other tumor-like lesions, benign and malignant neoplasms, should always be considered in the differential diagnosis of a hand mass. Clinical presentation of soft tissue sarcomas vary and may result in misdiagnosis and/or inappropriate treatment. An index of suspicion that any mass may be malignant, particularly a lesion that is found in an atypical location, should be entertained prior to surgical exploration to avoid widespread contamination.

The most common soft tissue sarcomas involving the hand include epithelioid sarcoma, rhabdomyosarcoma, and synovial sarcoma. Epithelioid sarcoma may be seen as a wart-like small lesion grossly and can histologically mimic granulomatous disease because of its characteristic central necrosis surrounded by chronic inflammatory cells with interspersed malignant epithelial cells. Rhabdomyosarcoma is most commonly seen in childhood and should be suspected if rapid growth is seen. Synovial sarcoma has an inconsistent behavior in that growth may be minimal for months to years and may then be followed by an accelerated burst of growth. Irregular calcifications on plain radiographs should raise a high index of suspicion that the lesion may represent synovial sarcoma.

Angiosarcoma, although exceedingly rare, nearly always occurs in older patients (>25 years) and has a predilection for superficial soft tissues and skin. Chronic lymphedema and radiation may be an etiologic factor leading to angiosarcoma. His-tologic, ultrastructure, and immunostains are important in diagnosing such tumors. Prognosis is generally poor and proximal extension of the disease is not uncommon even after wide exclusion.

Preoperative evaluation should include plain radiographs prior to surgical excision. If the lesion reveals calcification or if the mass is in an atypical location for common benign lesions of the hand, MRI should be obtained. Incisional versus excisional biopsy of the mass is determined by the index of suspicion and whether excision would lead to more significant contamination than an incisional biopsy. Staging includes local (size, depth, grade) and systemic evaluation (lymph node evaluation and computed tomography scan of the chest) to determine prognosis.

Treatment varies with each malignancy about the hand. Epithelioid sarcoma requires radical resection and is often resistant to chemotherapy and radiation therapy. Rhabdomyosarcoma responds well to chemotherapy and radiotherapy and may require only local excision. However, the vast majority of soft tissue sarcomas including angiosarcoma require wide excision, and clear margins must not be sacrificed for function.

Unfortunately, patients with angiosarcoma associated with chronic lymphedema may have multifocal disease, and radical amputation may be required.

Suggested Readings

Aygit AC, Yildirim AM, Dervisoglu S. Lymphangiosarcoma in chronic lymphoedema. Stewart-Treves syndrome. J Hand Surg [Br] 1999;24B: 135-137.

Bhaskaranand K, Navadgi BC. Glomus tumour of the hand. J Hand Surg [Br] 2002; 27B:229-231.

Chung KC, Kim HJ, Jeffers LL. Lymphangiosarcoma (Stewart-Treves syndrome) in postmastectomy patients. J Hand Surg [Am] 2000;25A:1163-1168.

Jablon M, Horowitz A, Bernstein DA. Magnetic resonance imaging of a glomus tumor of the fingertip. J Hand Surg [Am] 1990;15A:507-509.

Johnson DL, Kuschner SH, Lane CS. Intraosseous glomus tumor of the phalanz: a case repot. J Hand Surg [Am] 1993;18A:1026-1028.

Kline SC, Moore JR, deMente SH. Glomus tumor originating within a digital nerve. J Hand Surg [Am] 1990;15A:98-101.

Marcove RC, Charosky CB. Phalangeal sarcomas simulating infections of the digits: review of the literature and report of four cases. Clin Orthop 1972;83:224-231.

Prat J, Woodruff JM, Marcove RC. Epithelioid sarcoma. An analysis of 22 cases indicating the prognostic significance of vascular invasion and regional lymph node metastasis. Cancer 1978;41:1472-1487.

Theumann NH, Goettmann S, Le Viet D, et al. Recurrent glomus tumors of fingertips: MR imaging evaluation. Radiology 2002;223:143-151.

Wong CH, Chow L, Yen CH, Ho PC, Yip R, Hung LK. Uncommon hand tumours. Hand Surg 2001;6:67-80.

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