Intra-lesional interleukin-2 for the treatment of in-transit melanoma.

PURPOSE: To investigate the role of intra-lesional interleukin-2 (IL-2) injection for treatment of in-transit melanoma metastases. METHODS: Consecutive patients with in-transit metastases were treated with intra-lesional IL-2 injections. Two independent observers evaluated response to treatment using the Response Evaluation Criteria in Solid Tumors (RECIST) criteria. A blinded pathologist confirmed clinical response with post-treatment biopsies. RESULTS: Thirty-nine patients were included. Patients received biweekly IL-2 injections. At each treatment session, a mean of 2.08 ml (5 µ/ml) of IL-2 were distributed amongst a mean of 12 (range 1-57) in-transit lesions. Patients were followed for an average of 30.4 months (range 2.2-66.6 months). The overall patient response rate was 82%. A complete response was obtained in 20 patients (51%), a partial response in 12 (31%), and no response in seven (18%). Of the 629 in-transit metastases, 479 (76%) completely resolved. Complete responders had a significant in transit-free (P = 0.0005) and an overall (P = 0.012) survival advantage compared with partial responders. CONCLUSIONS: The treatment of in-transit metastatic melanoma with intra-lesional IL-2 resulted in a 76% percent clearance of lesions. Complete response is associated with superior in transit-free and overall survival when compared with partial response.

Atrophy among mucosa-only versus muscular mucosa superiorly based pharyngeal flaps: a pilot animal study.

BACKGROUND: Velopharyngeal insufficiency is frequently managed surgically via the superiorly based pharyngeal flap procedure. The flap used in this procedure traditionally consists of both mucosa and muscle. Although muscle is included to potentially increase survival and tissue bulk, its inclusion may result in postoperative dysfunction of pharyngeal sidewall motion. Although the benefits of pharyngeal flap surgery are established, the denervated muscular portion of the flap appears to atrophy. The purpose of this study was to evaluate whether a flap consisting entirely of mucosa provides the same tissue bulk as a conventional pharyngeal flap 12 weeks postoperatively. METHODS: Ten laboratory-grade cats served as direct patient surrogates. Cats were randomized to undergo a superiorly based pharyngeal flap procedure with flaps composed of either both muscle and mucosa (n = 5) or mucosa only (n = 5). Blinded volumetric analysis was performed by three board-certified neuroradiologists using magnetic resonance imaging of the pharynx immediately after surgery and at 4, 8, and 12 weeks. RESULTS: At 12 weeks postoperatively, pharyngeal flaps composed of both mucosa and muscle atrophied to reach volumetric equivalence with mucosa-only flaps. Evaluation of final pharyngeal flap volume demonstrated no statistical differences between the two groups. CONCLUSIONS: This study demonstrates equivalent final superiorly based pharyngeal flap tissue bulk for flaps composed of only mucosa when compared with conventional pharyngeal flaps. Thus, the mucosa-only pharyngeal flap may yield similar outcomes while potentially providing for an earlier return of normal pharyngeal function.

Malignant neoplasm of perivascular epithelioid cells of the liver.

Neoplasms of perivascular epithelioid cells (PEComas) have in common the coexpression of muscle and melanocytic immunohistochemical markers. Although this group includes entities with distinct clinical features, such as angiomyolipoma, clear cell sugar tumor of the lung, and lymphangioleiomyomatosis, similar tumors have been documented in an increasing diversity of locations. The term PEComa is now generally used in reference to these lesions that are not angiomyolipomas, clear cell sugar tumors, or lymphangioleiomyomatoses. While most reported PEComas have behaved in a benign fashion, malignant PEComas have occasionally been documented. We present a case of hepatic PEComa with benign histologic features, which nonetheless presented with metastases to multiple sites nearly 9 years later. This case represents the second documented malignant PEComa of the liver, as well as the longest follow-up of a surviving patient with a malignant PEComa, emphasizing both the need for criteria that more accurately predict the behavior of PEComas and the necessity of long-term follow-up of patients with PEComas.

Primary PEComa of the bladder treated with primary excision and adjuvant interferon-alpha immunotherapy: a case report.

BACKGROUND: Perivascular epithelioid cell tumors (PEComas) are rare mesenchymal neoplasms of uncertain malignant potential, which have in common the co-expression of muscle and melanocytic immunohistochemical markers. CASE PRESENTATION: A 48-year-old man presented with dysuria, passage of urinary sediment and lower abdominal discomfort. A three centimeter mass was identified by cystoscopy in the posterior midline of the bladder. Computerized tomography suggested an enterovesical fistula. The patient underwent laparotomy, partial cystectomy and partial small bowel resection. Pathological examination revealed PEComa of the bladder. The patient underwent adjuvant interferon-alpha immunotherapy. Subsequent follow-up procedures, including cystoscopy and imaging, have not revealed evidence of recurrence. The patient is clinically free of disease 48 months after surgery. CONCLUSION: This case represents the second documented PEComa of bladder and demonstrates that adjuvant therapies, including anti-angiogenic and immunotherapy, may be considered for patients with locally advanced or metastatic genitourinary PEComa.

Collagen-rich tumors of soft tissues: an overview.

Although relatively common, soft tissue tumors frequently present diagnostic problems for practicing pathologists. Immunohistochemistry has facilitated the diagnosis of many mesenchymal tumors; however, there is considerable overlap in the staining profiles among cells demonstrating fibroblastic and myofibroblastic differentiation. It has been our experience that soft tissue tumors associated with abundant extracellular collagen deposition commonly cause problems in classification. One reason for this is that tumors displaying this morphology include representatives from different histogenetic families. Specifically, tumors exhibiting fibroblastic, myofibroblastic and even lipomatous differentiation may manifest as a densely collagenous mass. It is the purpose of this review to highlight these collagen-rich soft tissue tumors.