Angiosarcoma of the of Scalp with Calvarium: DISCUSSION

21 Jan
2010

angiosarcoma cancer

Angiosarcoma of the scalp is a rare and deadly malignant tumor classically found in elderly Caucasian males. It was first described by Livingston and Klemperer in 1926, when they reported a tumor of the scalp in a 38-year-old white male who later died from uncontrolled hemorrhage from the tumor. E. Wilson Jones, 38 years after the first case was published, described a series of nine cases occurring on the face and scalp of elderly patients.

These tumors are more commonly found in the elderly with the mean age of presentation from 68-76 years. The male-to-female ratio is roughly 2:1.u 5,H,i9,23 They account for 1% of all sarcomas and less than 0.1% of all head and neck cancers. These tumors may be found in any location, although they have a predilection for the skin and superficial soft tissues in sharp contrast to most other sarcomas that are usually located deep within the body. Of those occurring on the skin and superficial soft tissue, over 50% are found in the head and neck region. In reference to the head and neck region, these lesions are frequently found in the upper half of the face or scalp. cialis canadian pharmacy

Figure 2. Skin ulceration with subjacent

Figure 2. A. Skin ulceration with subjacent granulation tissue (original magnification X40). B. High power of A showing granulation tissue with mononuclear chronic inflammatory cells (original magnification X250). C. Vascular channels dissecting through collagen (original magnification X250). D. Atypical cells lining the vascular channels (original magnification X250).

The rarity and masquerading presentation of angiosarcoma often lead to late diagnosis. Common presentations in the literature include induration, erythematous nodules, bruise-like plaques, fungating masses, ulcerations, alopecia, violaceous nodules, macules, and bleeding wounds. While the ulcerated and hemorrhagic lesion suggests a more-advanced stage of angiosarcoma, the indurated and plaque-likelesions are more indicative of poorly differentiated and moderately differentiated tumor, respectively.

Figure 3. Atypical endothelial

Figure 3. A. Atypical endothelial cells with hyperchromatic nuclei and an atypical mitotic figure in the center (original magnification X400). B. Lesion with granulation tissue-like appearance invading the calvarium (original magnification XI00). C. Diffuse and strong positive staining reaction with antibody against CD31, a marker for endothelial cells (original magnification XI00).

The aggressive nature of angiosarcoma is evident by frequent local invasion into the underlying calvarium and even brain invasion. Distant metastases occur up to 50%, via hematogenous as well as lymphatic route. The lung is the most common site of distant metastasis, followed by the liver. Other reported sites of metastases include heart, spleen, intestines, retroperitoneum, bone, bone marrow, and kidney. Delayed recurrence of angiosarcoma at distant sites has been reported, making regular lifelong surveillance paramount. The highly metastatic nature of this tumor has been postulated by Tanioka and colleagues to be due to the absence of vascular endothelial cadherin (VE-cadherin), which is present in normal endothelium. viagra soft

Figure 4. Tl-weighted MR image

Figure 4. A. Tl-weighted MR image showing tumor invasion through the outer table into the diploic portion of the skull, A. Coronal view and B. Sagittal view.

There are no delineated risk factors for this deadly, but chronic lymphedema, which was first reported by Treves and Stewart, and prior radiation exposure have been linked to angiosarcoma. McConnell and associates suggest connective tissue dystrophy caused by persistent, chronic lymphedema may be the inciting factor for the genesis of angiosarcoma. Also, the concept of connective tissue damage has been postulated by several based on the occurrence of angiosarcoma in adult patients who received radiation during infancy. Sun exposure and consequent actinic skin damage was once proposed to be associated with the development of angiosarcoma due to its rarity in blacks and overt predominance in Caucasians. But this theory has been refuted by other reports that showed most of the patients with angiosarcoma of the scalp had hair, affording protection to the affected sites. Trauma was also considered but not substantiated as an inciting agent for the development of angiosarcoma. Reed and colleagues, who reported a series of six cases in whom five had a history of minor trauma, concluded that the trauma may have “merely alerted the patient to the presence of the tumor rather than having served as a causative agent.” Other predisposing factors that have been cited in individual cases include occurrence of tumor at former herpes zoster sites, complication of telangiectatic nevus, other vascular and lymphatic abnormalities, and fistula complicating chronic osteomyelitis. In other reports, including the two largest series of angiosarcoma affecting the face and scalp, no underlying predisposing factors were found. tadalis sx

Figure 5. Intra-operative photograph

Figure 5. Intra-operative photograph showing excision of the involved calvarium.

The treatment of this dreadful, unrelenting sarcoma includes surgical excision with wide margins, radiation therapy, immunotherapy, and chemotherapy. A combination of any of these forms of treatment can be used with some success. With the exception of wide surgical excision, no single treatment modality has proven to be effective. Suecessful treatment with recombinant interleukin-2 (rIL-2) has been reported. Ulrich and associates advocate intralesional cytokine therapy alone or in combination with surface irradiation as an alternative therapy for nonsurgical candidates. Liposomal doxorubicin has been given with some success.

Radical electron-beam therapy of small lesions using wide fields affords the best opportunity of eradicating cutaneous lesions. cheap viagra uk

The overall prognosis of angiosarcoma is grim. It is an aggressive tumor with a high propensity for both local occurrence and distant metastases. The single most important prognostic factor is the size of the lesion at the time of presentation, making early diagnosis crucial. The histologic differentiation of the tumor has no effect on prognosis. However, a marked lymphocytic response and lack of appendageal destruction by the tumor have been associated with a better prognosis. Factors like age, sex of the patient, the location of the lesion (scalp vs. face), and the clinical appearance of the lesion have no statistically significant effect on the prognosis. The only statistically significant predictor of prognosis is tumor size at initial presentation. Lesions smaller than 5 cm have been shown by different groups to significantly increase the probability of survival. Some series reported a five-year survival of less than 10%. Others, including some of the biggest reviews up to date, cite a five-year survival of less than 30%. In the UCLA review, they reported 50% of the patients dying within 15 months. Maddox and colleagues reported a median survival of 20 months. In a recent review of prognostic factors for soft-tissue sarcomas using a relatively large database (1,240 patients), the following were found to be statistically significant independent predictors of subsequent metastasis: tumor size, tumor grade, neurovascular or bone involvement, and tumor depth. All of these factors, with the exception of tumor grade, are dependent on the tumor size. Hence, the need for early diagnosis and aggressive management with tumor excision cannot be overemphasized.

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