Microscopically controlled surgical excision combined with ultrapulse CO2 vaporization in the management of a patient with the nevoid basal cell carcinoma syndrome.

Nevoid basal cell carcinoma syndrome is an autosomal dominant condition characterized by multiple basal cell carcinomas, skeletal abnormalities and sometimes mental retardation. The large number of tumors, which are often disfiguring, presents extreme difficulties in the treatment of these patients. Microscopically controlled excision, compared to other modalities (radiation therapy, photodynamic therapy, intralesional interferon alpha-2b) offers the highest cure rate. However, because of the large size and involvement of wide areas of the skin, this approach is sometimes impractical. The ultrapulse CO2 laser with high energy and short pulses achieves char-free ablation of the tumors, bloodless surgical field, minimal nonspecific thermal damage, rapid healing and diminished postoperative pain. Also, a number of lesions can be removed in a single session. We present a 48-year-old man with a 6.5 x 4.5 cm large basal cell carcinoma involving the anterior abdomen and navel area. The central thick portion of the tumor was resected by microscopically controlled excision with 3 stages, and wide thinner peripheral crescentic plaque vaporized with ultrapulse CO2 laser. The laser settings were 300 mJ energy/pulse and 100 W average power, which corresponds to the fluence of 7.5 J/cm2. Computerized pattern generator (ultrascan handpiece) was adjusted to patterns of 3 (circle) and 1 (square) with sizes varying from 5 to 7, and density of 9 (60% overlapping). The tumor was vaporized with 6 passes, all the way to deep reticular dermis. A fifteen month-follow up disclosed no recurrent disease. Subsequent biopsies revealed only a scar with postinflammatory hyperpigmentation. Our experience indicates that combined treatment with microscopically controlled excision and ultrapulse CO2 laser ablation is a suitable modality for the large tumor plaques involving concave and convex areas of the skin respectively. Microscopically controlled excision of thicker, concave portions of basal cell carcinoma plaques, where CO2 laser surgery is less feasible, presents an effective addition that renders this combined modality a successful method for the treatment of nevoid basal cell carcinoma syndrome.

The use of antidesmoglein stains in Mohs micrographic surgery. A potential aid for the differentiation of basal cell carcinoma from horizontal sections of the hair follicle and folliculocentric basaloid proliferation.

BACKGROUND: Histopathologic differentiation between benign and malignant tissue is of utmost importance for the Mohs surgeon. Folliculocentric basaloid proliferation (FBP) shares many histologic features with basal cell carcinoma (BCC). It is most commonly associated with tumors of areas with abundant hair follicles such as nasal and perinasal skin. Residual BCC incorrectly identified as a horizontally sectioned hair follicle undoubtedly increases the risk of tumor recurrence. Excision of additional layers of normal tissue to remove "funny looking follicles" may have profound impacts on tissue conservation, preservation of function, and cosmesis. Electron microscope studies of BCC revealed a significant reduction of desmosomes compared with normal basal cells and hair follicle keratinocytes. OBJECTIVE: This study has assessed the potential of rapid staining with monoclonal antidesmoglein antibody (33-3D) to discriminate between BCC, horizontally sectioned hair follicles, and FBP. METHODS: A rapid immunoperoxidase technique with 33-3D antidesmoglein antibody was performed on Mohs frozen sections. We selected 18 patients with BCC of nasal and perinasal locations where histologic discrimination between residual tumor and tumor-free margins with FBP or horizontally sectioned hair follicle was equivocal. RESULTS: Fourteen sections disclosed the preservation of desmoglein marker delineating the cell membranes ("perimembranous" pattern) consistent with normal hair follicles. The sections were identified as tumor-free and no additional stages were performed. The remaining four sections revealed absent perimembranous pattern but presence of diffuse cytoplasmic staining. These were diagnosed as positive for residual BCC requiring the excision of another layer of tissue to obtain tumor-free margins. A follow-up period ranging from 6 to 24 months revealed no instance of recurrent disease. CONCLUSION: Rapid detection of desmoglein with 33-3D antibody is a promising tool for discrimination between residual BCC and FBP or horizontally sectioned hair follicles. It may enhance the sensitivity of Mohs surgery by disclosing the hidden foci of BCC, thus preventing tumor recurrence and unnecessary excision of normal tissue.

Multiple acral syringomata with uniform involvement of the hands and feet.

We present a case of a 43-year-old black woman with multiple syringomata located on the dorsa of the hands and feet. Although syringoma is a common adnexal tumor, acral presentation is very rare. Symmetrical involvement of the distal lower extremities has not been reported previously. This interesting clinical presentation should be included in the differential diagnosis of a papular eruption of the hands and feet.

Dermatofibrosarcoma protuberans treated with Mohs surgery. A case with CD34 immunostaining variability.

BACKGROUND: Immunohistochemical stains for CD34 antigen can help in differentiating dermatofibrosarcoma protuberans (DFSP) from other fibrohistiocytic tumors and in demarcating its surgical margins during Mohs surgery. However, variable expression of CD34 antigen in nodular areas can sometimes result in negative staining of tissue sections creating diagnostic and therapeutic dilemmas. OBJECTIVE: To show the variable expression of CD34 antigen in a DFSP tumor. METHODS: Case presentation of DFSP with immunohistochemical staining using the ABC peroxidase macromolecular complex. RESULTS: Microscopic examination of paraffin-embedded sections showed CD34-negative DFSP tumor cells with positive staining of endothelial cells. On frozen sections at the time of surgery the tumor cells stained strongly positive for CD34. CONCLUSION: This case demonstrates the variable expression of CD34 antigen by DFSP tumors and emphasizes the need to interpret with caution the results of these immunostains during Mohs surgery.

Time course of endothelial dysfunction and myocardial injury during coronary arterial occlusion.

The time course of the effects of permanent myocardial ischemia without reperfusion on the coronary vascular endothelium and myocardium were investigated in anesthetized cats. The left anterior descending (LAD) coronary artery was occluded for 1.5, 3.0, 4.5, or 6.0 h. Coronary rings from the ischemic LAD and the nonischemic left circumflex (LCX) arteries were tested for their responsiveness to the endothelium-dependent vasodilators acetylcholine (ACh, 0.1-100 nM) and the calcium ionophore A23187 (1-1,000 nM), and the endothelium-independent vasodilator sodium nitrite (NaNO2, 0.1-100 microM). Vasorelaxation was not significantly impaired in response to ACh after 1.5 h of ischemia and only moderately impaired after 3.0 h of ischemia (63 +/- 5% of control). However, after 4.5 h of ischemia the ACh-induced response was decreased to 33 +/- 4% of control and further declined to 31 +/- 4% of control after 6.0 h (P less than 0.001 from 1.5 h). There was no significant decrease in LCX ring vasorelaxant responses to vasodilators at all times, and the LAD rings only showed a moderately decreased response to NaNO2 after 6.0 h of ischemia (82 +/- 4% relaxation, P less than 0.05). Transmission electron microscopy revealed very little endothelial damage at 4.5 and 6.0 h, with only some subendothelial swelling noted. Damage to the myocardium did not become significant until after 4.5 h of ischemia, and cardiac myeloperoxidase activity, indicative of neutrophil accumulation, was not significant at any time.(ABSTRACT TRUNCATED AT 250 WORDS)

Neutrophil-mediated vasoconstriction and endothelial dysfunction in low-flow perfusion-reperfused cat coronary artery.

We investigated the interaction between activated cat polymorphonuclear neutrophils (PMNs) and coronary vascular endothelial cells in vitro. It was shown that 1) 90 minutes of low-flow perfusion without reperfusion had no deleterious effects on endothelium-dependent vasodilation, whereas 90 minutes of low-flow perfusion and 20 minutes of reperfusion with a blood cell-free solution induced a 20-25% endothelial dysfunction; 2) activated PMNs produced endothelium-dependent vasoconstriction in coronary artery rings isolated from cat hearts undergoing 90 minutes of low-flow perfusion and 20 minutes of reperfusion with a blood cell-free Krebs-Henseleit solution; 3) addition of the superoxide free radical scavenger, superoxide dismutase (150 micrograms/ml), or an antibody directed against CD18 of PMN adherence glycoprotein complex (MAbR15.7, 20 micrograms/ml) attenuated PMN-induced vasoconstriction significantly, but addition of a hydroxyl radical scavenger [N-(2-mercaptopropionyl)-glycine, 150 micrograms/ml], a cyclooxygenase inhibitor, or a lipoxygenase inhibitor had no protective effect; 4) exposure of rings to a superoxide radical-generating system (i.e., xanthine and xanthine oxidase) produced significant vasoconstriction that was similar to that observed with activated PMNs and was inhibited by superoxide dismutase; and 5) activated PMNs produced a marked coronary endothelial dysfunction characterized by a decreased response to the endothelium-dependent vasodilators acetylcholine and A23187. Addition of either superoxide dismutase or MAbR15.7 protected against endothelial dysfunction. These results indicate that activated PMNs produce significant vasoconstriction and endothelial dysfunction in coronary arteries isolated from low-flow perfusion-reperfused hearts. These effects appear to be mediated primarily by superoxide radicals generated by activated PMNs that either inactivate or inhibit the synthesis and release of endothelium-derived relaxing factor. We conclude that activated PMNs are able to induce endothelial dysfunction by releasing free radicals and possibly other substances.

Daltroban, a thromboxane receptor antagonist, protects the myocardium against reperfusion injury following myocardial ischemia without protecting the coronary endothelium.

The effects of daltroban, a specific thromboxane receptor antagonist, were investigated in a model of myocardial ischemia consisting of 1.5 h of coronary artery occlusion followed by reperfusion for 4.5 h in anesthetized cats. Daltroban (1 mg/kg) was infused as a bolus 10 min prior to reperfusion of the left anterior descending (LAD) coronary artery. Daltroban infusion resulted in a significantly lower necrotic area expressed as a percentage of the myocardial area-at-risk compared to the MI + vehicle group. However, daltroban failed to retard increases in myeloperoxidase activity in the ischemic myocardium, indicating no reduction in neutrophil accumulation. Moreover, left anterior descending coronary artery ring preparations isolated from daltroban treated MI cats exhibited endothelial dysfunction following ischemia reperfusion. Thus, daltroban significantly protected the myocardium from reperfusion injury without protecting the coronary endothelium or retarding neutrophil accumulation.