Clinical and Histopathologic Characteristics of Metastatic and Locally Advanced Cutaneous Basal Cell Carcinomas.

ABSTRACT: Locally advanced or metastatic basal cell carcinomas (laBCCs or mBCCs) are rare, with few case series providing information on their epidemiology. We aimed to describe the clinical and histologic features of locally advanced and metastatic basal cell carcinomas. Forty cases of laBCC or mBCC were identified by searching Vanderbilt's database from 1984 to January 2019. A retrospective chart review was performed. Pathology slides were available for 23 cases (13 mBCCs and 10 laBCCs). Twenty-one of 23 cases were Clark level IV or V, with a mean depth of invasion of >7 mm for both types. The mean mitotic rate was 4.4 mitoses/mm2 for laBCCs and 3.3 mitoses/mm2 for mBCCs. Ulceration was identified in 7 laBCC and 8 mBCC cases. Perineural invasion was present in 2 laBCC and 6 mBCC cases, with 3 mBCCs invading nerves >0.1 mm. Of 13 mBCC cases, histologic subtypes included infiltrative (n = 9), nodular (n = 7), morpheaform (n = 4), and superficial (n = 2), with multiple patterns present in some specimens. 10 of 13 patients with mBCC had local recurrence before metastasis. In summary, we identified several potential markers of high-risk BCC, including perineural invasion, deep invasion, elevated mitotic rate, and local recurrence of the primary tumor.

Cutaneous Metastases From a Cribriform Adenocarcinoma of the Minor Salivary Glands.

Cutaneous metastases from solid tumor malignancies often emanate from breast, gastrointestinal, and lung tumors. Adenocarcinomas from minor salivary gland cancers may involve the skin contiguously but rarely as metastatic deposits. Furthermore, these metastases are usually locoregional and not distal. Recently, an uncommon neoplasm termed cribriform adenocarcinoma of the minor salivary glands has been described, and although it often spreads to cervical lymph nodes, metastatic involvement of the skin has not been reported.

An atypical pleomorphic lipomatous tumor arising on the cheek.

Pathologists and dermatopathologists commonly encounter tumors with adipocyte differentiation. Most are of minimal clinical significance. Those exhibiting atypical spindle cell morphology have been reported but the terminology for such neoplasms is unsettled. Tumors with both spindle cell and pleomorphic morphology are rare, with equally unsettled descriptive nomenclature. Recently, a series of such tumors, termed atypical pleomorphic lipomatous tumor, has been published. They resided in the deep soft tissue or subcutis. To date, such a tumor has not been reported with dermal involvement.

Pancreatic fat necrosis in a 10-year-old girl: A case report and review of the literature.

We report here a case of a young girl with pancreatitis and pancreatic fat necrosis (PFN). This condition is rare in the pediatric age group, and its etiopathogenesis is different from disease in adults. Whereas PFN in adults typically results from pancreatitis secondary to pancreatic duct obstruction, alcohol abuse, and pancreatic adenocarcinoma, in children it appears to arise in a setting of systemic disease, often involving a genetic disorder.

In utero development of epidermolysis bullosa acquisita.

We report the case of an infant born with perioral vesicles that rapidly spread to involve his mouth and the majority of his body. Histopathology, immunofluorescence, and enzyme-linked immunohistochemistry assays confirmed a diagnosis of epidermolysis bullosa acquisita (EBA). His mother had no history of EBA, and serum indirect immunofluorescence was negative. The patient improved rapidly with local wound care and oral dapsone.

Risk Factors for Basal Cell Carcinoma in Men Younger Than 40 Years: A Case-Control Study.

BACKGROUND: Basal cell carcinoma (BCC) is the most common malignancy in the United States and is more prevalent in older populations. OBJECTIVE: The aim of this study was to investigate BCC risk factors in male patients younger than 40 years. MATERIALS AND METHODS: A consecutive series of male patients with pathology-proven BCC and younger than 40 years at time of diagnosis were retrospectively identified along with matched controls. Phone interviews were conducted using a structured questionnaire, and differences between patients with and without BCC were investigated. RESULTS: A total of 50 patients with BCC and 27 controls were included in this study. Compared with controls, patients with BCC worked outdoor jobs for longer lengths of time (43.2 vs 15.6 months; p = .04), were more likely to have a family history of skin cancer (66% vs 44%; p = .02), and were more likely to use sunscreen heavily after biopsy (p = .02). Patients with multiple BCCs (n = 20) were more likely to have a history of substantial recreational sun exposure (p = .01) than patients with solitary lesions (n = 30). CONCLUSION: The authors conclude that outdoor sun exposure in patients with underlying genetic susceptibility is the most likely mechanism of BCC formation in young male patients.

Pulmonary Signet-Ring Cell Adenocarcinoma Metastatic to the Skin.

Internal malignancies occasionally metastasize to the skin, usually imparting a poor prognosis. In men, pulmonary malignancies are the second most common offending tumor. Roughly, half such lesions are adenocarcinomas. A small subset of pulmonary adenocarcinomas exhibits signet-ring morphology. To date, a cutaneous metastasis arising from a pulmonary signet-ring cell adenocarcinoma has not been reported.

Imaging mass spectrometry assists in the classification of diagnostically challenging atypical Spitzoid neoplasms.

BACKGROUND: Previously, using imaging mass spectrometry (IMS), we discovered proteomic differences between Spitz nevi and Spitzoid melanomas. OBJECTIVE: We sought to determine whether IMS can assist in the classification of diagnostically challenging atypical Spitzoid neoplasms (ASN), to compare and correlate the IMS and histopathological diagnoses with clinical behavior. METHODS: We conducted a retrospective collaborative study involving centers from 11 countries and 11 US institutions analyzing 102 ASNs by IMS. Patients were divided into clinical groups 1 to 4 representing best to worst clinical behavior. The association among IMS findings, histopathological diagnoses, and clinical groups was assessed. RESULTS: There was a strong association between a diagnosis of Spitzoid melanoma by IMS and lesions categorized as clinical groups 2, 3, and 4 (recurrence of disease, metastases, or death) compared with clinical group 1 (no recurrence or metastasis beyond a sentinel node) (P < .0001). Older age and greater tumor thickness were strongly associated with poorer outcome (P = .01). CONCLUSIONS: IMS diagnosis of ASN better predicted clinical outcome than histopathology. Diagnosis of Spitzoid melanoma by IMS was strongly associated with aggressive clinical behavior. IMS analysis using a proteomic signature may improve the diagnosis and prediction of outcome/risk stratification for patients with ASN.

Syphilitic gumma arising in association with foreign material.

Until recently the number of patients with syphilis has been diminishing. Although that trend has reversed cases of tertiary syphilis are rare and often difficult to diagnose as a substantial number of patients will have a negative rapid plasma reagin. Histologically, cutaneous lesions in late stage syphilis exist in two forms, noduloulcerative and gummatous. Silver stains for spirochetes are almost invariably negative and, surprisingly, immunohistochemical stains are problematic as most lesions contain few, if any organisms. Presented here is a case of gummatous tertiary syphilis arising in association with foreign material deposited after a motor vehicle accident.

Intradermal nevi with atypical nuclei in the elderly: The senescent nevus.

BACKGROUND: Benign melanocytic nevi removed from elderly patients may demonstrate focal areas with nuclear atypia. OBJECTIVE: We sought to determine the prevalence of these nevi and their clinical and histologic features. METHODS: Intradermal nevi from patients older than 60 years were evaluated for areas of focal nuclear atypia and analyzed for 9 histologic characteristics. The patients' sex, biopsy sites, and clinical diagnoses were also tabulated. A statistical analysis of the 2 groups was undertaken. RESULTS: In all, 197 specimens from 157 patients were found. Twenty exhibited nuclear atypia (senescent nevi) and 177 did not (benign nevi). Significant differences were found for suggested clinical diagnosis, epithelioid-appearing melanocytes, the number of mast cells, evidence of solar elastosis, the number of intranuclear pseudoinclusions, and the presence of abnormally staining connective tissue. LIMITATIONS: This is a single-site, retrospective analysis involving a modest number of specimens. In addition, only a single age group was evaluated and only intradermal nevi were examined. CONCLUSIONS: The presence of focal nuclear atypia in benign melanocytic nevi in the elderly is not a rare finding. As these features are likely a result of the age of the lesion and possibly of long-standing ultraviolet light exposure, the term "senescent" nevus is suggested.

Munchausen syndrome and Munchausen syndrome by proxy in dermatology.

Patients with Munchausen syndrome purposefully injure themselves, often with the injection of foreign materials, to gain hospital admission and the attention associated with having a difficult-to-identify condition. Munchausen syndrome by proxy occurs when a child's caregiver, typically the mother, injures the child for the same reasons. Cases of Munchausen syndrome and Munchausen syndrome by proxy with primary cutaneous involvement appear to be rarely described in the literature suggesting either that diagnosis is not made readily or that it is, in fact, an uncommon disorder. At the center of both conditions is significant psychological pathology and treatment is difficult as many patients with Munchausen syndrome when confronted with these diagnostic possibilities simply leave the hospital. Little is known about the long-term outcome or prognosis of these patients.

Cutaneous Erysipelothrix rhusiopathiae (erysipeloid) infection in an immunocompromised child.

Erysipeloid, a cutaneous infection with the gram-positive bacillus Erysipelothrix rhusiopathiae, is typically an occupational dermatosis seen in persons working with livestock or involved in commercial fishing (fishmongers). Other more-generalized forms of infection with this organism also exist, including a septic form usually associated with endocarditis. Many infections may be self-limited. They have rarely been reported in children or in immunocompromised patients. This microbe is sensitive to many mainstream antibiotic agents.

Linear, pruritic red to brown papules on the left chest.

A 28-year-old woman presented with a 12-year history of red to brown papules in a linear distribution on the left lateral chest associated with recent flares of pruritus. She had previously been clinically diagnosed with lichen planus. A punch biopsy was performed, and histopathologic exam revealed Darier-like acantholysis. The patient was diagnosed with type 1 segmental Darier disease and her symptoms improved with topical tretinoin.

Mycobacterium smegmatis in skin biopsy specimens from patients with suppurative granulomatous inflammation.

Formalin-fixed, paraffin-embedded skin biopsy specimens, including 72 suppurative granulomatous inflammation (SGI) and 47 non-SGI controls, were tested for mycobacteria by using a broad-range PCR and a suspension array identification system. Mycobacterium smegmatis was detected in 13 (18.1%) of the SGI skin biopsy specimens, which was significantly more than 2 (4.3%) in the controls (odds ratio, 5.73; 95% confidence interval, 1.21 to 27.06; P = 0.028).

Cutaneous verrucous xanthoma in a bone marrow transplant recipient with recessive dystrophic epidermolysis bullosa.

Verrucous xanthomas (VXs) typically arise on the oral mucosa but have been reported on glabrous skin in patients with graft-versus-host disease or recessive dystrophic epidermolysis bullosa (RDEB). The inciting cause of these tumors is unclear but may result from cutaneous inflammation and trauma. We describe a patient with RDEB who had previously undergone a bone marrow transplant for her condition and subsequently developed an VX on her neck.

Leflunomide in dermatology.

Leflunomide is an oral disease-modifying antirheumatic drug administered to patients with rheumatoid and psoriatic arthritis. This drug inhibits dihydroorotate dehydrogenase, an enzyme critical in the production of intracellular pyrimidines, and down-regulates tumor necrosis factor-alfa activity. Leflunomide has demonstrated usefulness in treating cutaneous psoriasis along with other dermatologic and rheumatologic conditions.

Medical missions: a therapeutic primer.

Trips abroad to underdeveloped countries for the purpose of providing dermatologic care (medical missions), while both exciting and gratifying, require planning and forethought. What do I bring? What conditions will I encounter? What medications will be available? This paper outlines my experiences on such trips over the past 15 years, providing an approach and formulary which facilitates the performance of daily medical dermatology clinics.

Chromosomal translocation-negative cellular extraskeletal myxoid chondrosarcoma in an adolescent female.

Extraskeletal myxoid chondrosarcoma (EMC) is a relatively uncommon soft tissue sarcoma that typically presents in adults of middle age and affects the proximal thigh and limb girdles. Initially believed to be a low-grade malignancy, long-term patient follow-up has shown a high incidence of local recurrence and metastatic spread. EMC is uniformly resistant to chemotherapy and radiation therapy. These tumors characteristically display fibrous septae with large aggregates of mucin populated by clusters and strands of oval cells exhibiting minimal mitotic activity. A more aggressive cellular subtype has also been defined and exhibits basaloid cells showing the immunohistochemical staining features of neuroendocrine differentiation calling into question their proposed cartilaginous lineage. Most, although not all, examples of EMC possess a unique balanced chromosomal translocation [t(9;22)(q22;q12)] between the EWSR1 and NR4A3 (previously termed TEC) genes. Pediatric and adolescent cases of EMC are rare, as only 15 have been reported and appear to follow a more aggressive clinical course. Reported herein is a case of an EMC arising in the thigh of a 15-year-old female and the first to undergo evaluation of chromosomal translocation.