Two Cases of Cutaneous Sarcomatoid Squamous Cell Carcinoma Resembling Cutaneous Giant Cell Tumor of Soft Tissue.

ABSTRACT: Cutaneous sarcomatoid squamous cell carcinoma is well-described with histology resembling pleomorphic undifferentiated sarcoma featuring collagenous or myxoid stroma with or without elements of keratinizing squamous carcinoma. This report presents 2 cases of dedifferentiated squamous cell carcinoma (SCC) composed of sheets of malignant mononuclear cells with malignant osteoclast-like multinucleated giant cells, extravasated blood, and hemosiderin resembling cutaneous giant cell tumor (cGCT). In the first case, an exophytic facial mass of a 96-year-old woman removed by shave showing extensive cGCT-like tumor but with microscopic elements of SCC in situ and positivity for cytokeratin 5/6 in the malignant spindle cells and SCC. The second case involved a 32-year-old man with a pedunculated penile mass removed by shave biopsy, displaying malignant cytology resembling cGCT, focal staining for cytokeratin AE1/AE3 and p63, and CD68 highlighting the osteoclast-like giant cells. Molecular analysis revealed CDKN2A, TP53, and TERT. Upon reexcision, case 2 showed focally invasive keratinizing SCC associated with differentiated penile intraepithelial neoplasia and lichen sclerosus. Skin specimens with an exophytic mass histologically resembling cGCT but with malignant cytology should be meticulously evaluated for elements of SCC. Molecular analysis, detecting mutations like H3F3 or HMGA2-NCOR2 fusion, can aid in distinguishing cutaneous sarcomatoid squamous cell carcinoma from GCT bone or GCT soft tissue.

Retrospective analysis shows the cost of Mohs surgery decreases when adjusted for medical inflation.

BACKGROUND: Mohs surgery (MS) is the gold standard for treating nonmelanoma skin cancers in cosmetically sensitive areas. OBJECTIVE: To investigate MS costs over time when adjusting for medical inflation while considering the perspective of patients, payers, and health care systems. METHODS: A retrospective claim analysis using data from the International Business Machines MarketScan Commercial Claims and Encounters Database from 2007 through 2019 was performed. A query of the database for any instance of a MS-specific Current Procedural Terminology (CPT) code in adults (17311, 17312, 17313, 17314, and 17315) was conducted. Aggregate data per claim regarding coinsurance, total cost, deductible, copay, and insurance payout were provided for each CPT code annually. RESULTS: The total adjusted cost per claim decreased significantly (P < .001) for 4 of the 5 MS-specific CPT codes between 2007 and 2019: 17311 (-25%), 17312 (-15%), 17313 (-25%), and 17314 (-18%). The patient's adjusted out-of-pocket expense increased significantly (P < .0001) for 4 of the 5 MS-specific CPT codes: 17311 (33%), 17312 (45%), 17313 (34%), and 17314 (43%). CONCLUSION: Among the 4 most used MS-specific CPT codes (17311, 17312, 17313, and 17314), the total cost per claim decreased and the patient's out-of-pocket expense increased from 2007 to 2019.

Mohs Micrographic Surgery Versus Wide Local Excision in the Treatment of Merkel Cell Carcinoma: A Systematic Review.

BACKGROUND: Merkel cell carcinoma (MCC) is an aggressive neoplasm with high rates of recurrences. Current guidelines recommend wide local excision (WLE) with 1 to 2 cm margins. However, Mohs micrographic surgery (MMS) offers a potential advantage over WLE because of its ability of sparing healthy tissue and assessing 100% of margins. OBJECTIVE: To systematically evaluate the surgical modalities for the treatment of MCC. MATERIALS AND METHODS: Eligible articles were identified using MEDLINE, Scopus, EMBASE, and Cochrane Library. All available studies investigating surgical treatment of MCC with WLE or MMS were considered. RESULTS: Forty studies met the inclusion criteria. Thirty-one studies described patients treated with WLE, 3 with MMS, and 6 with either WLE or MMS. Subgroup analysis of Stage I MCC showed recurrence rates similar in both surgical modalities with local recurrence rate of 6.8% for WLE versus 8.5% for MMS (p = .64) and a regional recurrence rate of 15.2% for WLE versus 15.3% for MMS (p = .99). CONCLUSION: Overall WLE cases were at a higher stage at presentation. Subgroup analysis showed that MMS is not inferior to WLE excision for the treatment of Stage I MCC and is a reasonable option for anatomic locations where tissue sparing is important.

Second-Intention Healing in Mohs Micrographic Surgery: A Single-Center Academic Experience.

OBJECTIVE: To describe the trends in second-intention healing (SIH) use at a single Mohs micrographic surgery (MMS) dermatology clinic and assess outcomes and patient satisfaction. METHODS: The authors conducted a single-center, retrospective study of patients who underwent MMS from November 2012 through November 2018. Data obtained for each patient included sex, age, tumor characteristics, number of MMS stages, final defect size, and postoperative complications. Patient satisfaction of SIH was retrospectively assessed by telephone survey. RESULTS: Providers used SIH in 22% of all MMS cases (n = 159/718). It was most commonly used for defects located on the nose, ear, temple, and periocular region. The average defect size and number of MMS stages for tumor clearance were 1.3 cm and 1.5 stages, respectively. Overall, low rates of postoperative complications were observed, and 95% of patients reported optimal or acceptable levels of satisfaction. CONCLUSIONS: This study supports the idea that SIH is a safe, effective alternative for wound management and may have broader practice indications than those traditionally proposed.

Differentiation of Basal Cell Carcinoma and Trichoepithelioma: An Immunohistochemical Study.

ABSTRACT: Basal cell carcinoma (BCC) is the most common malignancy of the skin. It is an epithelial neoplasm with origin in the precursor cells of the interfollicular epidermis. Even though it has low metastatic potential, delay in management may lead to local destruction and morbidity. In contrast, trichoepithelioma (TE) is a benign tumor originating from the outer root sheath of the hair follicle. Similar to BCC, TE tends to affect the head and neck region. Both neoplasms may exhibit clinical and histopathological similarities, making them prone to misdiagnosis. Multiple immunomarkers have been used to distinguish among these entities, but so far, no single agent or combination of agents appear to be neither sensitive nor specific enough to differentiate between them. This study was divided into 2 parts. First, 17 cases of BCC and 14 cases of TE were stained with androgen receptor and bcl-2. Then, 27 cases of borderline/equivocal of BCC and 13 cases of borderline/equivocal TE were stained with the same protocol. Sensitivity and specificity were calculated for each individual immunomarker and for the combination of them. Androgen receptor positivity was 100% specific for BCC and borderline/equivocal BCC, whereas bcl-2 diffuse staining pattern demonstrated a sensitivity of 82.4% for BCC and 88.9% borderline/equivocal BCC. When both immunomarkers were combined, the sensitivity for BCC decreased (70.6%) but the specificity remained high (100%). Similarly, the sensitivity for borderline/equivocal BCC was 55.6%, whereas the specificity was 100%. Although moderately sensitive, combining both immunomarkers showed an excellent specificity to discriminate between BCC and TE.

Folliculocystic and Collagen Hamartoma: A Distinct Hamartoma Associated With Tuberous Sclerosis Complex.

ABSTRACT: Tuberous sclerosis complex (TSC) is a neurocutaneous disease characterized by cutaneous and extracutaneous hamartomas. Dermatologic evaluation is critical for early diagnosis because mucocutaneous manifestations account for 4 of 11 major and 3 of 6 minor diagnostic criteria. Folliculocystic and collagen hamartoma (FCCH) is a recently described entity associated with TSC. We herein describe the case of a 28-year-old woman with a history of TSC who presented with a scalp lesion present since childhood. Physical examination revealed a solitary, well-circumscribed exophytic tumor over the occipital scalp measuring 9 × 8 cm and covered with comedones and cyst-like structures. Biopsy of the lesion demonstrated thickening of the collagen bundles throughout the dermis, concentric perifollicular and perivascular fibrosis, an increased number of dilated vessels, and keratin-filled cysts lined by the infundibular epithelium. Clinicopathologic correlation was diagnostic for FCCH. The patient was referred for surgical excision. In addition, we review 11 other cases of FCCH previously reported in the literature.

Treatment of erythrodermic psoriasis with biologics: A systematic review.

BACKGROUND: Biologic medications for plaque psoriasis have been used to treat erythrodermic psoriasis (EP). Since the guidelines for management of EP were published, new biologic medications have been approved for the treatment of plaque psoriasis. OBJECTIVE: To analyze the evidence of biologic medications in the treatment of EP based on response and tolerability. METHODS: A comprehensive search was conducted with the PubMed, Cochrane Library, Embase, and Scopus databases through December 31, 2018. Studies reporting 1 or more cases of EP, defined as >75% body surface area involvement, in patients aged ≥18 years treated with biologics were included. Baseline Psoriasis Area and Severity Index score, score improvement, and adverse events were documented. Adequate response to treatment was defined as Psoriasis Area and Severity Index ≥50. RESULTS: Included were 43 articles, yielding a total of 179 patients. Most patients responded at some point during treatment, with a higher level of evidence for infliximab, ustekinumab, ixekizumab, and guselkumab. Infection was the most common adverse event (n = 35). LIMITATIONS: Data are limited to case reports, case series, and uncontrolled studies. CONCLUSION: Patients with EP treated with biologics demonstrated positive responses and treatment was well-tolerated, with a weak recommendation and limited quality of evidence in favor of infliximab, ustekinumab, ixekizumab, and guselkumab.

Rare Variant of Agminated Spitz Nevi on a Hypopigmented Background and Segmental Distribution: Case Report and Review of Literature.

Spitz nevus is a type of melanocytic nevus that can arise as a solitary lesion or as multiple lesions either disseminated or agminated (grouped) in different skin backgrounds (eg, grossly normal, hyperpigmented, or hypopigmented). Agminated Spitz nevi have been rarely reported and are even rarer in a background of hypopigmented skin. We present the case of a 2-month-old girl with multiple, grouped, dome-shaped, red papules arising on a hypopigmented patch with a segmental distribution. Biopsy of 2 lesions showed findings characteristic of Spitz nevus, confirming the diagnosis. We also review 4 other cases of agminated Spitz nevi arising on hypopigmented skin reported in the literature.

Treatment of sebaceous carcinoma with Mohs micrographic surgery versus wide local excision: a systematic review.

Sebaceous carcinoma (SC) is a rare neoplasm affecting periocular and extraocular sites. If inadequately treated, it can recur and cause morbidity. Specific management guidelines have not been established. Wide local excision (WLE) has been traditionally used; however, Mohs micrographic surgery (MMS) can be advantageous because of complete margin assessment and tissue-sparing nature. This analysis aims to systematically review the surgical modalities used for the management of SC. Articles meeting eligibility criteria were identified using MEDLINE (via PubMed), Embase, Cochrane, and Scopus databases. All studies investigating surgical management of SC with WLE or MMS were considered. Seventy studies met inclusion criteria, including retrospective cohort studies, case series, and case reports. WLE was used in 32 studies, MMS in 29, and MMS and WLE in 9. Subgroup analysis showed that MMS has lower recurrence rates. For WLE, local, regional, and distant recurrence rates were 23.4%, 13.3%, and 11.0%, respectively, and for MMS, 6.8%, 4.3%, and 4.6%, respectively. Patients treated with WLE were more likely to have local recurrence than patients treated with MMS (P = 0.001). WLE cases were more likely to have a regional (P = 0.05) and distant recurrence (P = 0.001). Limitations of the study include heterogeneity of case reports, case series, and retrospective studies, variable follow-up times between the two groups, and large tumors included in the WLE category. In addition, disease-specific survival was not evaluated. MMS cases showed a superior outcome for local, regional, and distant recurrence, making it a good option for the management of SC.

Psoriatic Alopecia in a Patient With Crohn Disease: An Uncommon Manifestation of Tumor Necrosis Factor α Inhibitors.

Tumor necrosis factor α (TNF-α) inhibitors are used to treat multiple inflammatory diseases including rheumatoid arthritis, inflammatory bowel disease, and psoriasis, among others. This family of medications can cause various side effects, some as common as injection-site reactions and others as rare as the paradoxical induction of psoriasiform skin lesions. Alopecic plaques recently have been described as an uncommon adverse effect of the TNF-α inhibitors adalimumab and infliximab. We present the case of a 12-year-old girl treated with adalimumab for Crohn disease who developed an alopecic crusted plaque on the scalp 6 months after increasing the dose of the medication. Biopsies, special stains, and sterile cultures yielded a diagnosis of psoriatic alopecia secondary to TNF-α inhibitor. A literature review for similar cases found 24 additional patients presenting with similar findings, of which only 6 were part of the pediatric population.

Periorbital and Globe Injuries in Pediatric Orbital Fractures: A Retrospective Review of 116 Patients at a Level 1 Trauma Center.

STUDY DESIGN: Retrospective chart review of pediatric and globe injuries associated with orbital fractures. OBJECTIVE: Our study seeks to examine these injuries and their association with orbital fractures at our trauma center to gain a better understanding of how to approach pediatric patients with orbital fractures. METHODS: A retrospective review of all facial fractures in pediatric patients at an urban level 1 trauma center was performed for the years 2002 to 2014. Patient demographics were collected, as well as orbital fracture location, mechanism of injury, concomitant injuries, ophthalmologic documentation, imaging, and perioperative records. RESULTS: One hundred sixteen pediatric patients over a 12-year period sustained an orbital fracture. The orbital floor was the most commonly fractured orbital bone in our series (60%). Thirty-four (30%) of the pediatric patients with orbital fractures had documented periorbital and/or globe injuries at the time of presentation. The most common periorbital injury was entrapment related to orbital floor fractures. Significant eyelid lacerations were present in seven patients, with five of these patients had canalicular injuries and two had canthal malposition. Five pediatric patients presented with traumatic optic neuropathy. Two patients had ruptured globes requiring enucleation. CONCLUSIONS: Periorbital soft tissue and globe injuries associated with orbital fractures occurs in a substantial number of pediatric patients. There are no guidelines for treatment of these type of injuries in the pediatric population. Further research should be performed to better understand the appropriate management of periorbital injuries in conjunction with surgical management of the orbital fractures.