Angiosarcoma of the head and neck managed by a combination of multiple biopsies to determine tumor margin and radiation therapy. Report of three cases and review of the literature.

BACKGROUND: Cutaneous angiosarcoma (AS) is a rare, often multicentric vascular tumor of the head and neck region with a rather poor prognosis. The original clinical size of the tumor rarely correlates to the degree of microscopic tissue invasion. Treatment by surgical excision sometimes requires very wide excision. Treatment by radiation or electron beam appears less mutilating but its efficacy is not well documented. OBJECTIVE: To present our experience with a combined surgical delineation of tumor margins followed by radiation treatment. METHODS: We treated three patients with extensive AS of the scalp and face. Prior to radiation, in two cases the tumor margins were determined by grid-pattern punch biopsies. In the third patient, the tumor margins were determined by Mohs mapping system. All three patients then received radiation either by rotational arc electron beam (n = 2) and standard radiation. RESULTS: One patient developed two local recurrences in nonirradiated areas plus a metastatic cervical node, all of which responded to additional electron beam. The patient has no evidence of disease (NED) after 30 months of observation. The other two patients were treated by electron beam and radiation have NED at 5 and 1 years follow-up, respectively. CONCLUSIONS: Local control of AS of the scalp may be achieved by assessment of the tumor margin by peripheral biopsies or Mohs technique followed by electron beam and radiation.

Bacillary angiomatosis: presentation of six patients, some with unusual features.

Bacillary angiomatosis (BA) is an unusual systemic vascular proliferation seen predominantly in patients with the acquired immunodeficiency syndrome. These vascular lesions are probably due to infection with a Bartonella species, most often B. henselae and, in some patients, B. quintana. BA is treatable and often curable, but without therapy, may be life-threatening. Clinically, the lesions, when superficial, are said to often resemble pyogenic granulomas, appearing polypoid histologically with an epidermal collarette. We now report six patients, three of whom showed lesions of BA morphologically and histologically distinct from the other patients reported to date. Two patients lesions appeared clinically as violaceous plaques and tumours resembling Kaposi's sarcoma; one of them had lesions histologically reminiscent of a papular angiokeratoma; and the other had lesions histologically suggestive of a combination of Kaposi's sarcoma and BA. Another patient presented with soft subcutaneous nodules which histologically showed extensive acute inflammation characteristic of an acute abscess, but which also displayed proliferating dilated small blood vessels with bulbous endothelial cells adjacent to numerous bacteria and also containing them. The Grocott-methenamine silver stain and the Warthin-Starry stain showed the organisms to better advantage in lesions of all six patients, although bacteria were also evident with the haematoxylin and eosin, periodic acid-Schiff and alcian blue stains.

Multiple agminated Spitz nevi: report of two cases and review of the literature.

Spitz nevus is most commonly a benign solitary lesion. Multiple lesions arranged in clusters (agminated) are very rare. Malignant degeneration has not been reported. Although spontaneous involution may occur, the nevi are frequently treated by surgical excision. We describe two preschool girls with multiple agminated Spitz nevi, and review the current world literature on multiple Spitz nevi.

Microcystic adnexal carcinoma (sclerosing sweat-duct carcinoma) of the head and neck.

We report 7 cases of microcystic adnexal carcinoma (MAC) that involved the face (n=6) and neck. The neck case occurred in a 10-year-old boy. MAC are tumors that typically extend far beyond their assessed clinical margins. This report emphasizes the need for early microscopically controlled excision of lesions because of the unreliability of clinical assessment and frequent perineural and bone extension. Treatment results of 35 cases treated by Mohs surgery are tabulated.

Protracted superficial Wegener's granulomatosis.

We describe a patient with protracted superficial Wegener's granulomatosis. On the basis of nasal mucosa and conjunctival biopsy specimens, a diagnosis of sarcoidosis was originally made. However, later biopsy specimens and the clinical condition indicated a rare variant of Wegener's granulomatosis, one with a protracted course of granulomatous ulcers localized to the skin and mucosa.

Q-switched ruby laser treatment of nevus of Ota.

Emission of 694-nm laser energy from a Q-switched ruby laser causes photodestruction of cutaneous pigment. The 40-nanosecond pulse duration of Q-switched ruby laser light initiates specific damage to melanosomes thus allowing selective treatment of benign pigmented lesions. Nevus of Ota is a benign facial oculocutaneous melanosis that has melanosomes lying deeply within the dermis. We report the successful use of the Q-switched ruby laser in the treatment of two patients with the nevus of Ota.

Behçet's disease presenting as superior vena cava syndrome.

Behçet's disease is a multisystem disease with many systemic manifestations. Vascular thromboses with a predilection for the venous system are a well-recognized complication. We report on a patient with superior vena cava syndrome and review different hypotheses regarding the underlying pathogenesis.

Buschke-Löwenstein tumor: verrucous carcinoma of the anogenitalia.

Buschke-Löwenstein tumor is a rare type of anogenital squamous cell carcinoma with a distinctive clinical appearance. We present the case of a thirty-four-year-old man with this tumor, which was excised surgically. Human papillomavirus was detected in formalin-fixed, paraffin-embedded sections with a human papillomavirus 6/11 probe, but not with a human papillomavirus 16 or a human papillomavirus 18 probe.

Epidermolytic hyperkeratosis treated with etretinate.

Epidermolytic hyperkeratosis is an unusual type of ichthyosis that may be characterized in extreme cases by massive cutaneous involvement resembling a porcupine. We present such a patient who had a remarkable response to treatment with oral etretinate.

Acitretin plus UVB therapy for psoriasis. Comparisons with placebo plus UVB and acitretin alone.

UVB radiation is beneficial for the treatment of psoriasis vulgaris. Patients with recalcitrant disease, however, are slow to respond to UVB phototherapy with and without the use of coal tars or emollients. Etretinate and, more recently, acitretin have proved useful, but clinical improvement is slow when they are used as monotherapy in plaque psoriasis. Each drug also produces side effects, some of which are dose related. This study was designed to compare results of treatment with UVB combined with either acitretin (50 mg/day) or placebo to determine if psoriasis would respond faster and to less cumulative exposure to UVB and acitretin. The psoriatic disease cleared to a greater degree in patients treated with acitretin-UVB with fewer treatments and smaller amounts of UVB radiation than in patients treated with either placebo-UVB or acitretin alone.