Prognostic value of 18F-FDG PET/CT and MRI features in patients with high-risk and very-high-risk cutaneous squamous cell carcinoma.

OBJECTIVE: This study aimed to evaluate the prognostic value of 18F-fludeoxyglucose positron emission tomography/computed tomography (FDG-PET/CT) and MRI features in patients with high-risk and very-high-risk cutaneous squamous cell carcinoma (cSCC). METHODS: This study included 54 consecutive patients with surgically resected primary high-risk and very-high-risk cSCC who underwent pre-operative FDG-PET/CT and/or MRI. Among them, 14 patients (26%) had recurrences. We retrospectively reviewed the FDG-PET/CT (n = 34) and MRI (n = 48) and investigated the clinical significance and prognostic value of imaging features in cSCC. RESULTS: On FDG-PET/CT, the maximum standardized uptake value (SUVmax) of the primary tumor (13.0 ± 6.4 vs. 6.9 ± 5.3, p < 0.05) was higher in cSCC with recurrence than in cSCC without recurrence. On MRI, the maximum diameter of the lesion (46.8 ± 24.1 mm vs 30.4 ± 17.0 mm, p < 0.05) and the frequency of muscle/tendon/bone invasion (42% vs 11%, p < 0.05) were significantly greater in cSCC with recurrence than in cSCC without recurrence. In the univariate analysis, prognostic factors for recurrence were SUVmax of the primary tumor (p < 0.01), the maximum diameter of the lesion (p < 0.05), and depth of invasion (p < 0.05). The areas under the receiver operating characteristic curves of the SUVmax (0.78) were superior to those of the maximum diameter (0.71) and depth of invasion (0.60). CONCLUSION: SUVmax, maximum diameter, and depth of invasion were useful parameters for prognostic factors predicting recurrence in patients with high-risk and very-high-risk cSCC. ADVANCES IN KNOWLEDGE: SUVmax represents a prognostic factor.

Imaging findings of trichilemmal cyst and proliferating trichilemmal tumour.

PURPOSE: The purpose of this study was to evaluate computed tomography and magnetic resonance imaging of benign trichilemmal cysts and proliferating trichilemmal tumours. METHODS: Nineteen histologically confirmed cutaneous lesions with trichilemmal keratinisation (12 trichilemmal cysts and seven proliferating trichilemmal tumours) were enrolled. Among them, 10 lesions (six trichilemmal cysts and four proliferating trichilemmal tumours) were examined by computed tomography, while 13 lesions (eight trichilemmal cysts and five proliferating trichilemmal tumours) were examined by magnetic resonance imaging. Computed tomography and magnetic resonance imaging characteristics were retrospectively reviewed. RESULTS: Sixteen lesions (84%, 10 trichilemmal cysts and six proliferating trichilemmal tumours) occurred on the scalp. Lobulated margins were observed in five lesions (26%, three trichilemmal cysts and two proliferating trichilemmal tumours). With respect to computed tomography attenuation, calcification (>200 Hounsfield units) was observed in seven lesions (70%, five trichilemmal cysts and two proliferating trichilemmal tumours), hyperdense areas (≥80 and ≤200 Hounsfield units) in six (60%, three trichilemmal cysts and three proliferating trichilemmal tumours), and soft tissue density areas (<80 Hounsfield units) in nine (90%, five trichilemmal cysts and four proliferating trichilemmal tumours). On T1-weighted images, intratumoral hyperintensity was only observed in eight trichilemmal cysts but no proliferating trichilemmal tumours (100% vs. 0%, P<0.01). On T2-weighted images, hypointense rim and intratumoral hypointensity was observed in all 13 lesions (100%, eight trichilemmal cysts and five proliferating trichilemmal tumours), and linear or reticular hypointensity was observed in 10 (77%, six trichilemmal cysts and four proliferating trichilemmal tumours). CONCLUSION: Trichilemmal cysts and proliferating trichilemmal tumours predominantly occurred on the scalp with calcification, and usually exhibited linear or reticular T2 hypointensity. Intratumoral T1 hyperintensity may be a useful imaging feature for differentiating trichilemmal cysts from proliferating trichilemmal tumours.

Magnetic Resonance Imaging Characteristics of Poroma and Porocarcinoma.

PURPOSE: The purpose of this study was to evaluate magnetic resonance (MR) imaging findings of poroma and porocarcinoma. METHODS: Six patients (3 male, 3 female; age range, 40-84 years; mean age, 61 years) with histologically confirmed skin appendage tumors with apocrine and eccrine differentiation (2 poromas and 4 porocarcinomas) were enrolled. All patients underwent preoperative MR imaging and the MR images were retrospectively reviewed. RESULTS: The configurations were classified as pedunculated solid in 5 lesions and subcutaneous cystic with mural nodules in 1. Well-demarcated deep tumor margins and smooth skin surfaces were observed in all 6 lesions, and peritumoral fat stranding was observed in 2. In all 5 pedunculated solid lesions, T2-hyperintense foci, T1 hyperintensity, and homogeneous solid components were observed within the lesions. CONCLUSIONS: Poroma and porocarcinoma usually exhibited pedunculated solid homogeneous lesion. Intratumoral T2-hyperintense foci and T1 hyperintensity were observed in pedunculated solid lesions.

Imaging findings of cutaneous angiosarcoma of the scalp: Comparison with cutaneous squamous cell carcinoma.

PURPOSE: This study aimed to evaluate imaging findings of cutaneous angiosarcoma (cAS) of the scalp compared with those of cutaneous squamous cell carcinoma (cSCC). METHODS: This study included 15 patients with primary cAS and 10 with primary cSCC of the scalp. Seven patients with cAS and eight with cSCC underwent magnetic resonance imaging, and 11 patients with cAS and eight with cSCC underwent 18F-fluorodeoxyglucose-positron emission tomography/computed tomography imaging. Imaging findings for both pathologies were retrospectively reviewed and compared. RESULTS: All 15 cAS cases were elevated lesions with an obtuse angle, invading the subcutaneous fat tissue. Multiple lesions were observed in only five cAS cases (33%) and no cSCC cases. Maximum diameter-to-height ratio was significantly higher in cAS than in cSCC (3.3 ± 1.0 versus 2.3 ± 0.6; p < 0.01). On T2-weighted images, intratumoral hypointensity (86% versus 13%; p < 0.01) and mixed hyper- and hypointensity (71% versus 0%; p < 0.01) were observed more frequently in cAS than in cSCC. No significant differences were observed between cAS and cSCC regarding flow void (29% versus 25%; p = 0.656). Maximum standardized uptake values were marginally significantly lower in cAS than in cSCC (5.6 ± 3.1 versus 10.5 ± 6.6; p = 0.078). CONCLUSIONS: Cases of cAS of the scalp always exhibited flat elevated lesions with invasion of the subcutaneous fat tissue. Compared with cSCC, intratumoral hypointensity and mixed hyper- and hypointensity on T2-weighted images were more frequent in cAS. These findings will help with the differential diagnosis of cAS.

Outcome of combination therapy using BRAF and MEK inhibitors among Asian patients with advanced melanoma: An analysis of 112 cases.

BACKGROUND: As most clinical trials evaluating BRAF and MEK inhibitor combination therapy (B + Minh) have been conducted in Western countries, little is known about the effect of B + Minh among East Asian populations. MATERIAL AND METHODS: Data from patients with advanced melanoma treated using B + Minh (either dabrafenib + trametinib or encorafenib + binimetinib) were retrospectively collected from 16 institutes in Japan. Response rates, adverse events, patterns of failure and survival were analysed. RESULTS: We analysed 112 of 144 collected patient records and, of these, 14 had acral/mucosal melanoma. The response rate for the entire cohort was 75.0%. There were no statistical differences in response rates between acral/mucosal and cutaneous melanomas (64.3% versus 76.5%), whereas previous treatment using immune checkpoint inhibitors (ICIs) did not affect response (72.7% versus 73.9%) to B + Minh, response to ICI after B + Minh was only 20%. Patients who achieved complete response had the best overall survival rates at 24 months (94.7%). Elevated serum lactate dehydrogenase levels and 3 or more metastatic sites were independently associated with survival. The most common relapse site was the brain (17.9%). More than half of the patients (58.8%) experienced grade III/IV pyrexia. CONCLUSION: B + Minh was effective among Japanese patients with melanoma, including those with acral/mucosal melanoma. Factors associated with survival were similar to previous Western studies. B + Minh response was not affected by the previous use of ICI; however, vigilance against brain metastasis during B + Minh therapy is required as the brain was our most commonly encountered relapse site.

MR imaging findings for differentiating cutaneous malignant melanoma from squamous cell carcinoma.

PURPOSE: This study aimed to assess the efficacy of using MR imaging findings for differentiating cutaneous malignant melanoma (cMM) from cutaneous squamous cell carcinoma (cSCC). METHODS: Preoperative MR images of patients with histopathologically proven primary cMM and primary cSCC were retrospectively reviewed and compared between the two pathologies. RESULTS: A total of 16 patients with primary cMM (7 men and 9 women; age range, 45-97 years; median age, 75 years) and 49 with primary cSCC (37 men and 12 women; age range, 46-90 years; median age, 76 years) were enrolled in this study. Intratumoral T1 hyperintensity compared to that of the dermis was more frequently observed in cMM than in cSCC (50 % vs. 4 %; p < 0.01). Superficial depression (51 % vs. 19 %; p < 0.05), superficial irregular margins (55 % vs. 25 %; p <  0.05), and reticular or linear T2 hyperintensity (27 % vs. 0 %; p <  0.05) were more frequently observed in cSCC than in cMM, respectively. CONCLUSIONS: cMM predominantly exhibited intratumoral T1 hyperintensity, whereas cSCC predominantly exhibited superficial depression, superficial irregular margins, reticular or linear T2 hyperintensity.

Intensive granulocyte and monocyte adsorption apheresis for generalized pustular psoriasis.

Granulocyte and monocyte adsorption apheresis (GMA) is usually performed weekly (consisting of five sessions) for refractory skin diseases, such as generalized pustular psoriasis (GPP). The time to remission of inflammatory bowel diseases has been reported to be significantly shorter in intensive GMA (twice a week) than in regular GMA (once a week). Despite several reports of GPP cases treated with intensive GMA, the efficacy of intensive GMA has not been verified in GPP. Herein, we present two GPP patients with a mutation in the IL36RN gene, who initially received regular GMA, and intensive GMA upon recurrence. There were no adverse effects during regular and intensive GMA for both patients. Because concomitant medication was only prednisolone (20 mg/day) during regular and intensive GMA, intensive GMA showed superiority to regular GMA in patient 1. Although concomitant medications were different between regular and intensive GMA in patient 2, these drugs had been used before the start of each GMA therapy. We cannot neglect the effects of concomitant drugs, but we observed a shorter time to remission in intensive GMA than that in regular GMA in both patients. More case studies will be necessary for evaluating the clinical efficacy of intensive GMA.

A Case of Dermatomyositis Coexisting with Both Anti-Mi-2 and Anti-NXP-2 Antibodies.

Myositis-specific autoantibodies (MSAs) including anti-Mi-2 and anti-nuclear matrix protein 2 (NXP-2) antibodies have been detected in the patients with dermatomyositis (DM), and are useful tools for identifying clinical subsets of DM. MSAs are exclusively found in DM patients. Anti-Mi-2 antibody-positive DM patients show the typical skin lesions and myositis and are rarely associated with internal malignancy and interstitial lung disease (ILD). On the other hand, adult DM patients with anti-NXP-2 antibody often show calcinosis and internal malignancy, but rarely ILD. In addition, anti-NXP-2 antibody-positive DM patients have severe phenotype with myalgia, peripheral edema, and significant dysphagia, but with mild skin lesions. Herein, we report a rare case of classic DM coexisting with both anti-Mi-2 and anti-NXP-2 antibodies, clinically, without ILD or internal malignancy. Our patient had typical skin manifestations, muscle weakness, muscle pain, and general fatigue without calcinosis, peripheral edema, or dysphagia. Thus, the clinical phenotype was similar to anti-Mi-2 antibody-positive DM.

Magnetic Resonance Imaging Findings Differentiating Cutaneous Basal Cell Carcinoma from Squamous Cell Carcinoma in the Head and Neck Region.

OBJECTIVE: This study aimed to evaluate the efficacy of magnetic resonance (MR) imaging in differentiating between cutaneous basal cell carcinoma (cBCC) and cutaneous squamous cell carcinoma (cSCC) in the head and neck region. MATERIALS AND METHODS: Among patients with cutaneous head and neck cancers, 14 with primary cBCCs and 15 with primary cSCCs with a histologic tumor height of ≥ 4 mm underwent MR examinations; the findings were then examined for correlations. RESULTS: cBCCs (71%) occurred more frequently on the nose than cSCCs (13%) (p < 0.01). The maximum diameter (23.5 ± 7.2 mm vs. 12.7 ± 4.5 mm; p < 0.01) and diameter-to-height ratio (2.8 ± 0.9 vs. 1.7 ± 0.4; p < 0.01) were significantly greater in cSCCs than in cBCCs. Superficial ulcer formation (67% vs. 21%; p < 0.05), protrusion into the subcutaneous tissue (60% vs. 21%; p < 0.05), ill-demarcated deep tumor margins (60% vs. 7%; p < 0.01), and peritumoral fat stranding (93% vs. 7%; p < 0.01) were more frequently observed in cSCCs than in cBCCs. Intratumoral T2-hyperintense foci (57% vs. 13%; p < 0.05) were more frequently observed in cBCCs than in cSCCs. CONCLUSION: cBCCs predominantly occurred on the nose with intratumoral T2-hyperintense foci, whereas cSCCs predominantly exhibited a flattened configuration, superficial ulcer formation, protrusion into the subcutaneous tissue, ill-demarcated deep tumor margin, and peritumoral fat stranding.

Clinical and histopathological characteristics and survival analysis of 4594 Japanese patients with melanoma.

BACKGROUND: The incidence of melanoma among those of an Asian ethnicity is lower than in Caucasians; few large-scale Asian studies that include follow-up data have been reported. OBJECTIVES: To investigate the clinical characteristics of Japanese patients with melanoma and to evaluate the prognostic factors. METHODS: Detailed patient information was collected from the database of Japanese Melanoma Study Group of the Japanese Skin Cancer Society. The American Joint Committee on Cancer seventh Edition system was used for TNM classification. The Kaplan-Meier method and Cox proportional hazards model were used to estimate the impact of clinical and histological parameters on disease-specific survival in patients with invasive melanoma. RESULTS: In total, 4594 patients were included in this analysis. The most common clinical type was acral lentiginous melanoma (40.4%) followed by superficial spreading melanoma (20.5%), nodular melanoma (10.0%), mucosal melanoma (9.5%), and lentigo maligna melanoma (8.1%). The 5-year disease-specific survival for each stage was as follows: IA = 98.0%, IB = 93.9%, IIA = 94.8%, IIB = 82.4%, IIC = 71.8%, IIIA = 75.0%, IIIB = 61.3%, IIIC = 41.7%, and IV = 17.7%. Although multivariate analysis showed that clinical classifications were not associated with survival across all stages, acral type was an independent poor prognostic factor in stage IIIA. CONCLUSIONS: Our study revealed the characteristics of melanoma in the Japanese population. The 5-year disease-specific survival of each stage showed a similar trend to that of Caucasians. While clinical classification was not associated with survival in any stages, acral type was associated with poor survival in stage IIIA. Our result might indicate the aggressiveness of acral type in certain populations.

Classification of 3097 patients from the Japanese melanoma study database using the American joint committee on cancer eighth edition cancer staging system.

BACKGROUND: The American Joint Committee on Cancer (AJCC) 8th Edition Cancer Staging System was implemented in 2018; however, it has not been validated in an Asian melanoma population. OBJECTIVE: The purpose of this study was to validate the new system using a cohort of Japanese melanoma patients. METHODS: The AJCC 7th and 8th Editions were used for TNM classification of patients in a database established by the Japanese Melanoma Study Group. Patient data with sufficient information to be applicable to the AJCC 8th staging were selected. The Kaplan-Meier method was used to estimate disease-specific survival and relapse-free survival. RESULTS: In total, data for 3097 patients were analyzed. The 5-year disease-specific survival according to the 7th and 8th Edition staging system were as follows: IA = 98.5%/97.9%; IB = 95.4%/96.2%; IIA = 94.2%/94.1%; IIB = 84.6%/84.4%; IIC = 72.2%/72.2%; IIIA = 76.2%/87.5%; IIIB = 60.7%/72.6%; IIIC = 42.0%/55.3% and IIID = none/26.0%. The 5-year relapse-free survival according to the 7th and 8th Edition staging was as follows: IA = 94.5%/92.7%; IB = 85.4%/85.3%; IIA = 80.1%/79.4%; IIB = 71.4%/70.6%; IIC = 56.8%/55.7%; IIIA = 56.8%/69.4%; IIIB = 42.6%/56.8%; IIIC = 20.0%/33.3% and IIID = none/6.5%. CONCLUSION: The results show that new staging system could efficiently classify our Japanese melanoma cohort. Although there was no difference in Stage I and II disease between the 7th and 8th Edition systems, we should be careful in managing Stage III disease since the survival curves of the 8th Edition staging were completely different from the 7th Edition. Moreover, our results indicate that adjuvant therapies for Stage IIB and IIC should be developed, since the relapse-free survival for these stages were equivalent to Stage IIIA and IIIB, respectively.

Anti-MDA5 Antibody-Positive Dermatomyositis Presenting with Cellulitis-Like Erythema on the Mandible as an Initial Symptom.

Panniculitis is an uncommon skin eruption observed in patients with dermatomyositis (DM)/clinically amyopathic dermatomyositis (CADM), especially in anti-melanoma differentiation-associated gene 5 (MDA5) antibody-positive DM. We present here a 51-year-old Japanese woman with an anti-MDA5 antibody-positive DM who initially had cellulitis-like erythema on her right mandible. Histopathological findings showed a subcutaneous lobular infiltration of lymphocytes. The patient developed typical skin eruptions of DM/CADM, rapidly progressive interstitial lung disease, and severe muscle weakness 2 weeks after the first visit. After the diagnosis of anti-MDA5 antibody-positive DM, she was treated with intravenous steroid pulse therapy (methylprednisolone, 1,000 mg/day for 3 days), oral prednisolone at 1.0 mg/kg/day, and tacrolimus at 4.0 mg/day. The lesions of panniculitis associated with DM/CADM typically present on the buttocks, thighs, arms, and abdomen. This is the first DM/CADM case with localized panniculitis on the face. Panniculitis and myositis usually show simultaneous improvement during treatment. Although panniculitis disappeared with steroid and tacrolimus treatment and did not recur, muscle weakness was intractable and recurred in this case. This indicates that the clinical courses of panniculitis and myositis of DM/CADM do not always change in parallel.

Enhanced dendritic cells and regulatory T cells in the dermis of porokeratosis.

Porokeratosis is characterized clinically by annular lesions and histologically by the presence of a cornoid lamella (CL) in the epidermis. The underlying mechanism of porokeratosis development remains unclear. We performed immunohistochemical staining of CD1a, langerin, Ki67, CD3, CD4, CD8, FOXP3, and RANKL (receptor activator of nuclear factor κB ligand) in samples from 17 porokeratosis lesions and analyzed the differences in staining patterns among the CL, the inner part of the annular ridge (IC), and the adjacent normal skin (ANS). Numbers of CD1a+ Langerhans cells in the epidermis were reduced and numbers of CD1a+ dermal dendritic cells were significantly increased in the CL and IC compared to those in the ANS. In addition, there was also an increase in FOXP3+ cells in the dermis below the CL and IC. Our findings suggest that Langerhans cells are downregulated in the epidermis in CL and that regulatory T cells and dendritic cells are upregulated in the dermis below the CL. This alteration in the distribution of immune cells, such as various lymphocyte subsets, Langerhans cells, and dermal dendritic cells, may play a key role in the pathomechanisms of porokeratosis.