Assessing smoldering adult T cell leukemia/lymphoma: a case report.

Adult T cell leukemia/lymphoma (ATLL) is a rare, extremely aggressive malignancy with four different clinical variants, all of which are associated with human T cell lymphotropic virus type 1. Antiretrovirals have been recognized as an effective therapy after study in clinical trials around the world. However, oncologists traditionally wait for asymptomatic patients with ATLL to reach a more severe stage of disease before treatment is initiated. We describe a patient with Fitzpatrick Skin Type V who presented with asymptomatic dyschromia of her neck, breast, and jaw. Her clinical, laboratory, and histological findings were consistent with smoldering type-ATLL. We teamed up with her oncologist to successfully treat her cutaneous symptoms with interferon-alpha/zidovudine. This report demonstrates efficacy with the employment of antiretroviral therapy immediately following a diagnosis of smoldering type ATLL.

An immunohistochemical panel to assess ultraviolet radiation-associated oxidative skin injury.

Ultraviolet (UV) radiation results in a significant loss in years of healthy life, approximately 1.5 million disability-adjusted life years (DALYs), and is associated with greater than 60,000 deaths annually worldwide that are attributed to melanoma and other skin cancers. Currently, there are no standardized biomarkers or assay panels to assess oxidative stress skin injury patterns in human skin exposed to ionizing radiation. Using biopsy specimens from chronic solar UV-exposed and UV-protected skin, we demonstrate that UV radiation-induced oxidative skin injury can be evaluated by an immunohistochemical panel that stains 8-hydroxydeoxyguanosine (8-OH-dG) to assess DNA adducts, 4-hydroxy-2-nonenal (HNE) to assess lipid peroxidation, and advanced glycation end products (AGEs) to assess protein damage. We believe this panel contains the necessary cellular biomarkers to evaluate topical agents, such as sunscreens and anti-oxidants that are designed to prevent oxidative skin damage and may reduce UV-associated skin aging, carcinogenesis, and inflammatory skin diseases. We envision that this panel will become an important tool for researchers developing topical agents to protect against UV radiation and other oxidants and ultimately lead to reductions in lost years of healthy life, DALYs, and annual deaths associated with UV radiation.

Microcystic adnexal carcinoma of the vulva.

BACKGROUND: Microcystic adnexal carcinoma (MAC) is a subset of sweat gland carcinoma first described as a specific entity by D. J. Goldstein, R. J. Barr, and D. J. Santa Cruz (Cancer 1982;50:566-72). We report the first case of MAC occurring on the vulva and review the literature pertaining to this rare tumor. CASE: A 43-year-old multiparous black woman presented initially to Kings County Hospital Medical Center with a chief complaint of a vulvar lesion arising on the left labia majora which she had noted for 4 years prior to presentation. Aside from increasing paresthesia in the area, she denied any constitutional symptoms. Her past medical history was significant only for hyperthyroidism and mild hypertension and surgical history was noncontributory. Gynecologic history was unremarkable, with sporadic care over the last 20 years. Physical examination revealed a 1.5 x 2.0-cm raised, well-circumscribed, firm mobile lesion on the left labia majora. It was noted to be yellow in color with the surrounding tissue being unremarkable in character. The remainder of her gynecologic examination and lymph node survey was unremarkable. Preoperative chest X ray was negative as was the CAT scan of the abdomen and pelvis. All laboratory values were within normal limits. A Pap smear done preoperatively was significant for atypical squamous and glandular cells of undetermined significance. Subsequent colposcopic examination of the cervix was remarkable for cervicitis and was adequate, with the entire transformation zone visualized. Both endocervical curettage and endometrial biopsy were normal. Initially, an excisional biopsy was performed with final pathology demonstrating microcystic adnexal carcinoma with positive surgical margins. She subsequently underwent a left radical hemivulvectomy with bilateral inguinal groin lymph node dissection. At the time of surgery, the left labia majora was noted to be well healed, with a residual surgical scar easily discernible. No areas of discoloration were noted and digital palpation of the area was unremarkable. Microscopic residual tumor was noted; however, all surgical margins and lymph nodes were negative for tumor. Her postoperative course was unremarkable. The patient has continued to do well since the time of her surgery and is being followed conservatively. CONCLUSION: Radical vulvectomy should be performed when MAC occurs in the vulva to secure negative margins of resection. Groin dissection should be reserved for cases in which the inguinal lymph nodes are clinically suspicious or in cases of tumor recurrence.

Outer root sheath acanthoma.

The term "outer root sheath acanthoma" is proposed to specify a distinctive keratosis exhibiting outer root sheath differentiation. This lesion features anastamosing columns of keratinocytes emanating from the undersurface of the epidermis that respectively demonstrate, with progressive descent into the upper dermis, outer root sheath differentiation characteristic of the infundibulum, isthmus, stem and anagen bulb. In our patient, the lesion was solitary, and no associated systemic disease was evident. Outer root sheath acanthoma is a unique keratosis displaying all four types of tricholemmal differentiation, thus representing a true neoplasm of the entire outer root sheath.

Eccrine syringofibroadenoma surrounding a squamous cell carcinoma: a case report.

A 91-year-old man presented with a 9.0 x 7.0 cm exophytic mass on the dorsum of the right foot, surrounded by a scaling hyperkeratotic plaque-like lesion that had been present for many years. He had similar long-standing hyperkeratotic plaque-like lesions on both legs. Histopathologic examination of the exophytic mass revealed a well-differentiated squamous cell carcinoma surrounded by an eccrine syringofibroadenoma (ESFA). Histochemistry, immunohistochemistry and electron microscopy support this diagnosis. To our knowledge, this is the only reported case of ESFA being intimately associated with a malignant neoplasm.

Papular mucinosis and human immunodeficiency virus infection.

Cutaneous mucin deposition occurs both as an isolated phenomenon and in patients with various systemic disorders. Among these are endocrinopathies (eg, hypo- and hyperthyroidism), malignancy (mycosis fungoides), connective tissue disorders (lupus erythematosus), and infectious diseases (scleredema associated with upper respiratory tract infection). We present a case of papular mucinosis in a patient infected with the human immunodeficiency virus. This case represents the third report of these disorders coexisting.

Volume of malignant melanoma is superior to thickness as a prognostic indicator. Preliminary observation.

There are many clinical and histologic factors that are known to be valuable in predicting survival rates for patients with cutaneous malignant melanomas. Breslow thickness is considered to be the most reliable prognostic factor; however, thickness is a unidimensional measurement. A more accurate mensuration to predict biologic behavior might be one that takes into account the three-dimensional volume of the neoplasm. In a study of 35 primary malignant melanomas, the volumes of the dermal components of the tumors were calculated. Those patients with tumor volumes of 200 mm3 or less had a 91.4% 5-year disease-free survival rate, compared with survival rate of only 16.7% for those patients whose lesions had tumor volumes exceeding 200 mm3. On multivariate analysis, tumor volume exceeded thickness as a prognostic indicator. Thus, measurement of tumor volume proved to be of greater significance than thickness in predicting the outcome for patients with malignant melanomas.

Distinguishing benign and malignant melanocytic lesions with the AgNOR method.

A silver staining technique has recently been devised to aid in the differentiation between benign and malignant melanocytic lesions. This study showed a statistically significant difference between the staining of silver-nucleolar organizer regions (AgNORs) in melanocytic nevi and that of AgNORs in malignant melanomas.

The histopathology of dysplastic nevi. Continued controversy.

The histopathologic criteria used in the diagnosis of dysplastic nevi have been a source of controversy, as has the clinical significance of these lesions. Several dermatopathologists noted for their work on dysplastic nevi were asked to contribute responses to questions regarding the architectural and cytological criteria used to classify a melanocytic nevus as dysplastic, the terminology used to describe these lesions, and the role of dysplastic nevi as precursors of melanoma. Although no consensus has been reached regarding the cytologic features required for diagnosis of dysplastic nevi, there is substantial agreement regarding the architectural features of these lesions.

Spitz nevi in black children.

Four black children with Spitz nevi are presented. The initial clinical diagnosis was pyogenic granuloma for three patients. One child had two Spitz nevi. Histologic examination revealed melanocytic dendritic hyperplasia in all cases.

Widespread flat warts associated with human papillomavirus type 5: a cutaneous manifestation of human immunodeficiency virus infection.

Numerous flat and tinea versicolor-like warts developed on the face, trunk, and upper extremities of a 10-year-old boy with human immunodeficiency virus infection. Nucleic acid analysis of involved skin revealed human papillomavirus type 5, which has sometimes been associated with epidermodysplasia verruciformis. This human papillomavirus type has also been described in patients with common variable immunodeficiency and dyskeratosis congenita and in renal allograft recipients. Human immunodeficiency virus infection should be added to the list of immune-related disorders that predispose to widespread flat warts.

Clinical characteristics of malignant melanomas developing in persons with dysplastic nevi.

A total of 452 patients with dysplastic nevi (DN) were followed prospectively by repetitive, complete cutaneous examinations in order to determine the clinical features of early malignant melanomas (MM) arising in them. Sixteen patients (3.5%) developed 18 newly diagnosed MM during an average follow-up period of 27 months. Twelve of the 18 MM were in situ and all of the primary invasive MM diagnosed prospectively in this follow-up were less than 0.89 mm in Breslow thickness, implying an excellent prognosis. The principal clinical clue to the diagnosis of MM was change in a preexisting pigmented lesion. Total-body photographs were very useful in helping to identify the early MM in these patients.

Dysplastic nevi. Markers for increased risk for melanoma.

A total of 452 white patients, classified into four dysplastic nevi groups, were followed prospectively by repetitive, complete cutaneous examinations using total-body photographs taken on entry into the study. Sixteen patients (3.5%) developed 18 newly diagnosed malignant melanomas (MM) during an average follow-up period of 27 months. Twelve of the 18 MM were in situ, and all of the six primary invasive MM diagnosed prospectively in this follow-up were less than 0.89 mm in Breslow thickness, implying an excellent prognosis. Compared with reference populations, the number of MM detected significantly exceeded the number estimated to occur in the comparable age-matched control groups. These data support the concept of repetitive follow-ups of all groups of patients with dysplastic nevi.

Disseminated warts and evolving squamous cell carcinoma in a patient with acquired immunodeficiency syndrome.

A patient with acquired immunodeficiency syndrome developed widespread verrucous lesions on the hands, feet, and penis. Histologic analysis revealed typical changes of verruca vulgaris and, from several sites, a transformation toward squamous cell carcinoma. Southern blot analysis of deoxyribonucleic acid from penile wart tissue indicated the presence of a type of human papillomavirus that rarely is associated with carcinoma.

The relationship between melanocytic nevi and malignant melanoma.

In conclusion, although there are data, some quite convincingly implicating dysplastic nevi and congenital nevi (particularly "giant") as "precursors" of malignant melanomas, our ability to predict the magnitude of these associations is lacking. Thus, until additional basic and clinical research data are forthcoming, any recommendation to prophylactically remove all congenital nevi or all dysplastic nevi in order to decrease the incidence of malignant melanoma is premature. In regard to congenital nevi, evidence exists that giant (larger than 20 cm in diameter) congenital nevi may have a significant risk factor so as to warrant, when feasible, prophylactic excision of such lesions. In our opinion, no uniform recommendation can be made at this time for the management of small and medium-sized congenital nevi. Patients with familial dysplastic nevus syndrome should be followed carefully and educated concerning the early detection of malignant melanoma. Patients with sporadic dysplastic nevus syndrome deserve further study to enable us to accurately determine their risk of developing malignant melanoma.

Congenital malignant melanoma.

A 6-week-old girl with congenital melanoma of the skin is reported and the literature is reviewed. We believe this to be the first case of congenital melanoma in a black infant who had no underlying giant melanocytic nevus. Because of the significant relationship between the early treatment and prognosis of malignant melanoma, we wish to alert pediatric clinicians to the existence of this rare congenital neoplasm.

Secondary syphilis presenting as a palisading granuloma.

A diagnosis of secondary syphilis was made in a 37-year-old man who presented with a papular eruption and a penile ulceration. Skin biopsy revealed a palisading granuloma similar to that seen in granuloma annulare. This represents a histologic finding that has not been described for early secondary syphilis.

The dysplastic nevus. Clinical and pathologic features.

The dysplastic nevus has not only been considered to be a "marker," but also a formal "precursor" of malignant melanoma. Therefore, these lesions are important to recognize clinically. This article presents a classification of the dysplastic nevus based upon its variable clinical presentations. It is hoped that this classification will assist the physician to recognize many of the clinical variants of this unusual melanocytic nevus and, thus, to identify patients at greater risk for the development of malignant melanoma.