Prognostic factors and evaluation of mycosis fungoides and Sézary syndrome.

BACKGROUND: Staging evaluations of patients with mycosis fungoides (MF) and Sézary syndrome (SS) are performed to individualize therapy and to predict survival. OBJECTIVE: Our purpose was to determine the prognostic factors in patients with MF and SS. METHODS: A retrospective study of 101 patients was performed. For inclusion in the study, patients had to have been evaluated for MF or SS within 6 months of the initial definitive histologic diagnosis. The evaluation included physical examination, chest radiograph, peripheral blood smear, lymph node biopsy, bone marrow biopsy, gallium 67 scan, liver-spleen scan and computed tomography (CT) of the chest, abdomen, and pelvis. RESULTS: The type of skin disease present at initial diagnosis was a good prognostic indicator of survival and clinical outcome. Univariate adverse prognostic features included hepatosplenomegaly or adenopathy by CT scan, abnormal liver-spleen scan, abnormal gallium scan, adenopathy, and peripheral blood, bone marrow, and lymph node involvement. Independent prognostic factors in multivariate analysis were the type of skin involvement as well as peripheral blood and visceral involvement. CONCLUSION: Our study confirms previous reports that type of skin and peripheral blood and visceral involvement are important prognostic factors in patients with MF or SS. Our results support the finding that patients with T1 stage disease have an excellent survival outlook and clinical outcome.

Clinical value of renovascular resistive index measurement in the diagnosis of acute obstructive uropathy.

PURPOSE: We evaluated the clinical use of the renal resistive index in identifying patients with acute urinary tract obstruction. MATERIALS AND METHODS: Of 54 patients with suspected acute urinary tract obstruction who underwent measurements of renal resistive index 19 had unilateral obstruction documented with excretory urography and comprise our study sample. The contralateral nonobstructed kidneys served as controls. Criteria for obstruction were a resistive index of 0.70 or greater or a side to side difference of 0.10 or greater. We calculated sensitivity, specificity, and positive and negative predictive values. RESULTS: Sensitivity for obstruction was 42% with 11 false-positive cases, specificity was 79%, and positive and negative predictive values were 67 and 57%, respectively. CONCLUSIONS: Renal resistive index measurements are not valuable in detecting acute urinary tract obstruction.

Photodynamic therapy in the treatment of Bowen's disease.

BACKGROUND: The treatment of Bowen's disease in anatomically difficult areas or especially large lesions can challenge accepted modalities of treatment. OBJECTIVE: The purpose of this study was to illustrate the effectiveness of photodynamic therapy in the treatment of Bowen's disease. In addition, photodynamic therapy may be used as adjuvant therapy for difficult lesions. METHODS: Six patients with Bowen's disease in various anatomic sites were treated with photodynamic therapy. Four were in a difficult anatomic site, or were especially large, or both. Photofrin, 1.0 mg/kg, was administered intravenously and laser treatment was given approximately 48 hours later with the argon dye laser. Light was administered at a wavelength of 630 nm and the light dose ranged from 185 to 250 joules/cm2. Treatment was given by surface radiation only. RESULTS: Eight lesions were treated. All showed a complete response at 3 months (100%) and continue to show a complete response at 6 and 12 months. Morbidity was low; the most significant side effects were moderate pain and edema. Healing time varied depending on the size of the lesion. CONCLUSION: Photodynamic therapy is an effective and useful alternative for Bowen's disease, especially those lesions in anatomically difficult areas or those that are especially large.

Increased prostaglandin E2 and cAMP phosphodiesterase levels in Kaposi's sarcoma--a virus against host defense mechanism.

In Kaposi's sarcoma tissue, prostaglandin E2 (PgE2) levels and cAMP phosphodiesterase levels were found to be higher than in surrounding normal tissue. We have shown earlier that PgE2 suppresses interferon (IFN) alpha production. High levels of cAMP phosphodiesterases result in low cAMP levels. Thus, this phenomenon may be involved in altered immunologic resistance, growth and differentiation.

Response to tumor necrosis factor in two cases of psoriasis.

A 70-year-old woman was treated for renal cell carcinoma with recombinant human tumor necrosis factor, 450,000 U/m2 of body surface area, daily for 5 days, once a month. She had had psoriasis for 25 years. After two courses of recombinant human tumor necrosis factor her psoriasis resolved. A 40-year-old man with severe psoriasis was subsequently treated with a total of seven complete and incomplete 5-day courses of recombinant human tumor necrosis factor at doses of 50,000 to 300,000 U/m2/day. Significant toxicity (fever, chills, hypertension, and hypotension) was encountered. Partial resolution of the lesions was seen. Tumor necrosis factor is an active agent in psoriasis.

Effect of splenectomy upon the growth of B16-F10 melanoma and its relation to the interferon system.

The influence of splenectomy upon the growth of B16-F10 malignant melanoma and changes in interferon-synthesizing ability in mice were studied. Surgical stress alone temporarily diminished the ability of mice to respond to interferon induction by poly rIrC. Two weeks following the surgery, mock-splenectomized mice fully regained their interferon synthesis ability. However, this was not true in the case of splenectomized mice. They remained refractory to interferon induction. The removal of the spleen had no obvious effect on the rate of pulmonary metastasis in mice injected with B16-F10 malignant melanoma in relation to the mock-splenectomized or control mice. Mice that were splenectomized and inoculated with B16-F10 melanoma also remained refractory to interferon induction.

Pigmented Bowen's disease and review of 420 Bowen's disease lesions.

Pigmented Bowen's disease is rare. We report an unusual case of pigmented Bowen's disease of the ring finger, which clinically presented as a superficial, spreading melanoma. Records of 420 lesions of Bowen's disease were reviewed; 7 lesions (1.67%) were pigmented and all occurred outside the anogenital area. We conclude that Bowen's disease should be considered in the differential diagnosis of pigmented lesions.

Granulomatous reactions in mycosis fungoides.

Granulomatous reaction characterized by the formation of noncaseating accumulations of epithelioid histiocytes and multinucleated giant cells of the foreign-body type is a rare, poorly understood, and generally ignored phenomenon seen in various types of lymphoma. Its presence in cutaneous infiltrates of mycosis fungoides is equally unusual, but some favorable prognostic significance has been ascribed to it previously. In this paper, four patients with typical mycosis fungoides and granulomas demonstrated histologically in their cutaneous infiltrates are presented. All four died of disseminated disease with evidence of central nervous system involvement in three of them. These clinical histories lend no support to the notion that granulomatous mycosis fungoides is a benign variant of this lymphoma. Relevant literature is reviewed and discussed.

Squamous cell carcinoma complicating late chronic discoid lupus erythematosus.

Two cases of squamous cell carcinoma developing in lesions of chronic cutaneous discoid lupus erythematosus are presented. One of the patients was white and the other was black. The squamous cell carcinoma in the black patient proved to be rapidly metastatic and eventually fatal. The white patient developed seven squamous cell carcinomas over 8 years with no evidence of metastatic spread. The role of ultraviolet light in inducing skin cancer in these patients is discussed.

Dysplastic nevus syndrome: association with multiple primary neoplasms.

A 65-year-old white man with dysplastic nevus syndrome is presented. The patient also developed an extramammary Paget's disease of the scrotum, two malignant melanomas of the skin of the arm and abdomen, two squamous cell carcinomas in the mouth, and several benign tumors such as lentigo maligna, dermatofibroma, and a cavernous hemangioma. Besides the well-established tendency of patients with the dysplastic nevus syndrome to develop malignant melanomas of the skin, their possible propensity to develop other primary malignant neoplasms is discussed.

Skin cancer caused by grenz rays.

A 30-year-old woman developed chronic radiodermatitis and five squamous cell carcinomas within 7 years following a 16-year period of repeated grenz irradiations given to her psoriasis. The estimated cumulative dose of grenz radiation was approximately 3,000 rads (r) to each treated area. This case, illustrating a rather underestimated carcinogenic potential of grenz rays, is interpreted in light of the available literature on the subject. The technical bases of grenz ray therapy are also briefly reviewed.

Paget's disease of the breast presenting as a cutaneous horn.

A 67-year-old woman developed a cutaneous horn on the nipple of her right breast. Biopsy of the skin underlying the horn disclosed Paget's disease of the breast. An intraductal adenocarcinoma of the same breast was found on mastectomy. High index of suspicion is mandatory in evaluating all nipple lesions.

Breast carcinoma metastatic to eyelids: case report and review of the literature.

Violaceous, indurated plaques around both eyes were found to be cutaneous metastases and the initial clinical presentation of a lobular carcinoma of the breast in an 80-year-old woman. Available literature indicates that breast carcinoma is the most common metastatic tumor of the eyelid and the onset of a palpebral mass may precede the diagnosis of the primary tumor in the breast.

Unusual clinical presentation of epidermotropic cutaneous lymphoma. Small hypopigmented macules.

A 35-year-old black woman developed numerous vitiligo-like small hypopigmented macules on the upper and lower extremities. Histologically the lesions were consistent with epidermotropic mycosis fungoides or pagetoid reticulosis. The patient is presented because of a clinical picture unusual for either of the above microscopic diagnoses.

Squamous cell carcinoma in dominant type epidermolysis bullosa dystrophica.

The unusual genetic disorder epidermolysis bullosa dystrophica has been reported in several patients in whom the chronic cutaneous scars led to the development of cutaneous squamous cell carcinoma. However, only one of these previously reported cases involved the autosomal dominant form of the disease; the remainder occurred in its recessive counterpart. We report the second and third patients with squamous cell carcinoma associated with the dominant form of epidermolysis bullosa dystrophica. In addition, we not only observed the previous electron microscopic findings of decreased numbers of anchoring fibrils beneath the basal lamina but have also noted marked disruption of the basal lamina itself.

Reticulate pigmented anomaly.

Reticulate pigmented anomaly of the flexures is a benign, recently described genodermatosis reported to involve the flexural areas as well as the scrotum. Described herein is a twenty-nine year old man in whom the characteristic speckled macules were limited to the scrotal and penile skin. The authors suggest that the anomaly be designated reticulate pigmented anomaly without reference to specific anatomic sites. It is possible that this pigmented eruption of the genitalia is a cutaneous marker of this patient's underlying testicular carcinoma.

Nonspecific antigen reactions.

Cell-mediated immune challenge reactions to a number of antigens at tumor sites resulted in regressions of various types of benign, premalignant, and malignant lesions in man. Regressions varied from partial to complete and lasted from several months to more than 10 years. Increased levels of cell-mediated immunocompetence were attained by several means, including reduction of tumor burden, immunopotentiation, or transfer of immunity. Antitumor activities of immune challenge reactions are selective for tumor cells and appear to be independent of the nature of the antigens used or the type of neoplasm. Combinations of immunotherapy with other treatment modalities resulted in augmentation of antitumor effects. Preliminary studies indicate that cellular and noncellular mediators of delayed hypersensitivity induce antitumor activities that may be significant in the regressions of neoplasms induced by cell-mediated immune challenge reactions. Since cell-mediated immune reactions are frequent occurrences, they may be a factor in apparently spontaneous regressions of neoplasms.