Lower Extremity Angiosarcoma: A Life-Threatening Complication of Lymphedema.

ABSTRACT: When angiosarcoma, a rare and aggressive tumor of the soft tissue, develops in the setting of chronic lymphedema, it is referred to as Stewart-Treves syndrome. It is usually seen in chronic lymphedema of the upper limbs postmastectomy. Angiosarcoma developing in the lower limb in the setting of chronic lymphedema is rare and has a poor outcome. The presentation of angiosarcoma can vary, ranging from a bleeding papule to a plaque or a subcutaneous mass, which can later progress to ulceration or necrosis. Treatment for Stewart-Treves syndrome is aggressive because of its poor prognosis and usually requires a multidisciplinary approach of surgery, radiation, and chemotherapy. Several theories have been put forth to explain the mechanism of Stewart-Treves syndrome, but it remains ambiguous. The current literature regarding angiosarcoma developing in the setting of chronic lymphedema in the lower limb is limited to single case reports. Herein, the authors report a series of six cases of biopsy-proven angiosarcoma in the setting of lower extremity lymphedema. Providers should include angiosarcoma in the differential diagnosis of ulcerative or vascular tumors arising in the context of lower extremity lymphedema.

Association of Lower Extremity Lymphedema and Nonmelanoma Skin Cancers.

OBJECTIVE: To determine whether patients with lymphedema of a lower extremity (LE) had a greater risk of skin cancer than those without lymphedema. PATIENTS AND METHODS: This retrospective cohort study included patients with LE lymphedema examined at Mayo Clinic in Rochester, MN, USA, from January 1, 2000, through December 31, 2020. All patients with the phrase "lower extremity lymphedema" and a diagnostic code for lymphedema present in their electronic health record, as well as their age-, race-, and sex-matched controls without lymphedema, were included in the study. A Kaplan-Meier curve was constructed to examine the time to development of the first skin cancer for the lymphedema cohort and the controls. A Cox proportional hazards regression model was used to calculate hazard ratios. RESULTS: In total, 4437 patients had lymphedema within the study period. Compared with the matched control group, the lymphedema group had a significantly increased risk of skin cancer. For the subset of patients with unilateral lymphedema, the lymphedematous extremity was 2.65 times as likely as the nonlymphedematous LE to have skin cancer, particularly basal cell carcinoma. CONCLUSION: Lower extremity lymphedema appears to be a risk factor for squamous cell carcinoma, basal call carcinoma, and as expected, angiosarcoma. Clinicians caring for patients with LE lymphedema should be aware of this increased risk and monitor at-risk patients accordingly.

Neutrophilic eccrine hidradenitis: a retrospective study.

BACKGROUND: Neutrophilic eccrine hidradenitis (NEH) is a benign neutrophilic dermatosis not well recognized beyond an association with malignancy. Although the disease is commonly reported in adults with malignancy, this association is uncommon in children. The diagnosis of NEH is predominantly based on histologic findings to exclude alternative diagnoses for adults, but biopsy is not usually required for children. METHODS: A retrospective study was performed of adult and pediatric patients diagnosed with NEH at three Mayo Clinic sites from January 1, 1992, to January 1, 2022. The aim of this study was to elucidate risk factors for NEH and its clinical characteristics, treatment options, and natural course. Clinical information and pathologic results were collected from health records. Available pathologic slides were reviewed with a dermatopathologist. RESULTS: Of 47 patients identified, 33 had either histologic or clinical confirmation of the diagnosis; 21 were adults (64%), and 12 were children (36%). Most adults (16/21; 76%) had underlying malignancy and received chemotherapy. Five adults (24%) were classified as having idiopathic NEH, and they were younger and had higher NEH recurrence rates than the other adults. Only one pediatric patient (8%) had underlying malignancy. For 10 children (83%), NEH was preceded by strenuous activity. Initial findings of idiopathic NEH were palmoplantar eruptions for both adult and pediatric patients, whereas malignancy-associated NEH commonly involved the face and axillae. CONCLUSIONS: Among adults, NEH is commonly associated with malignancy and chemotherapy. Among children, idiopathic NEH occurs primarily after overexertion, and malignancy is highly unlikely to be the cause of NEH.

Differences in Skin Cancer Rates by Transplanted Organ Type and Patient Age After Organ Transplant in White Patients.

Importance: Although it is known that patients with thoracic organ transplants develop skin cancer more frequently than those who receive nonthoracic organ transplants, patterns of risk for subsequent skin cancers are unknown. Objective: To further characterize organ transplant recipients who develop multiple skin cancers and assess for patterns of development of additional skin cancers beyond the first skin cancer diagnosis by patient age and transplanted organ type. Design, Setting, and Participants: This cohort study used validated electronic health record-based data from a single tertiary care academic medical center to identify 5129 solid organ transplant recipients who underwent transplant surgery between 1992 and 2017 and were older than 18 years at the time of transplant. The cohort was limited to White patients because they have the highest skin cancer risk based on phenotype. The mean follow-up was 6.6 years. Data were analyzed June 9, 2021, to May 31, 2022. Main Outcomes and Measures: Differences in rates of skin cancer development for first and subsequent skin cancers were measured using t test or analysis of variance and χ2 tests for continuous and categorical variables. Rates of skin cancer development were compared based on organ type and patient age at transplant using Fine-Gray tests and cumulative incidence plots. Results: A total of 5129 organ transplant recipients (mean [SD] age, 51.3 [12.9] years; 3287 men [64.1%]) were included. Of these, 695 patients (13.6%) had development of at least 1 skin cancer, with 6842 skin cancers identified in the cohort overall. Compared with liver transplant recipients, heart, lung, or kidney recipients were more likely to develop at least 1 skin cancer (χ2 test, 25.6; df, 4; P < .001). There was no significant difference by transplanted organ type in the rate of developing a second or third skin cancer; however, the age at transplant was associated with the time to developing a second (χ2 test, 20.4; df, 4; P < .001) or third (χ2 test, 10.9; df, 4; P < .02) skin cancer. Conclusions and Relevance: This cohort study found that there was no difference by organ type for development of subsequent skin cancers in organ transplant recipients, and recipients of all organ types developed additional skin cancers at high rates after the initial skin cancer.

Suggestions for a New Clinical Classification Approach to Panniculitis Based on a Mayo Clinic Experience of 207 Cases.

BACKGROUND: Panniculitis, or inflammation of adipose tissue, includes a heterogeneous group of disorders with similar morphologic presentations. Currently, panniculitides are classified based on histopathologic findings only. OBJECTIVE: In this retrospective study of 207 cases of biopsy-proven panniculitis over 20 years at Mayo Clinic, we aimed to propose a new classification that integrates the clinical morphologic features with the histopathology of panniculitis. METHODS: We collected patient demographic and lesion morphologic characteristics using lesion photographs and physician notes for each of our 207 cases, including location, ulceration, scale, pattern (unilateral versus circumferential), atrophy/sclerosis (cicatricial), redness, and swelling. RESULTS: The panniculitides most likely to ulcerate were calciphylaxis (85.7% ulcerating), pancreatic panniculitis (66.6%), and α1-antitrypsin deficiency-associated panniculitis (100%). The panniculitides least likely to ulcerate were erythema nodosum and medication-induced and granulomatous panniculitis. This retrospective study used only descriptions in clinical notes and available medical photographs. CONCLUSION: We present an updated classification schema of panniculitides based on clinical findings. The primary distinctions are based on ulceration, location, and number of lesions. Although complete distinction of all panniculitides based on clinical examination alone is not possible, we hope the proposed schema allows clinicians to tailor differential diagnoses.

Validity of Using Billing Codes From Electronic Health Records to Estimate Skin Cancer Counts.

IMPORTANCE: Patients can develop multiple skin cancers, and their medical data can be spread over multiple health care systems. This fragmented care, combined with the lack of skin cancer registries, has limited our ability both to provide accurate estimates of incidence and to study the pathogenesis of multiple skin cancers. OBJECTIVE: To assess whether standard diagnostic and procedural codes present in the electronic health records at a single health care system are a valid proxy for estimating the number of overall skin cancers. DESIGN, SETTING, AND PARTICIPANTS: Retrospective cohort study of patients seen at a single-center tertiary care hospital (ie, Vanderbilt University Medical Center) between July 1, 2008, and June 30, 2018. All patients with at least 1 electronic health record-based diagnostic or procedural code for any skin cancer and at least 1 pathology report of a skin cancer. EXPOSURE: The number of International Classification of Disease (ICD) or Current Procedural Terminology (CPT) codes relating to skin cancer. MAIN OUTCOMES AND MEASURES: Pearson correlation coefficient and R2 were calculated for the total number of ICD or CPT codes for skin cancer and histologically verified skin cancers. RESULTS: In this cohort study of 35 901 patients, the mean (SD) age was 70.4 (14.4) years, 20 404 (56.8%) were men, and 31 623 (88.1%) were White individuals. Of these patients, 6307 had at least 1 ICD or CPT code or pathology report for a skin cancer, of whom 5688 patients had both a CPT code related to skin malignancy and a histologically verified skin cancer. There was a strong linear correlation between the number of CPT codes and pathology records (r = 0.87). There was a poor correlation between the number of ICD codes and pathology records (r = 0.22). CONCLUSIONS AND RELEVANCE: This cohort study found that the use of ICD codes was a poor proxy measure for the number of skin cancers per patient. In contrast, CPT codes accounted for more than 75% of the variability in the number of skin cancers (R2 = 0.76) and were a better proxy measure for the total number of skin cancers per patient.