A Rare Case of Pseudo-Malignant Paranasal Extranodal Rosai-Dorfman Disease.

Rosai-Dorfman disease is a very rare disease characterized by histiocytic accumulation in the head and neck region and lymph node enlargement. We report a rare pseudo-malignant paranasal extranodal Rosai-Dorfman disease. A 69-year-old-man presented nasal bleeding and nasal obstruction. Paranasal mass was detected in the left nasal cavity and computed tomography (CT) findings are the sphenoid sinus, maxillary sinus, and ethmoid sinus were involved with inconstant bone thickening, however, no bone destruction was detected. Magnetic resonance imaging scans show iso-intensity signal in T1-weighed image and T2-weighed image. Positron emission tomography/CT fluorodeoxyglucose (FDG) uptake in posterior ethmoid sinus and sphenoid sinus, bilateral cervical lymph node, clavicle, and sternum. Based on the above results, we considered malignant lymphoma and performed a biopsy. After pathological examination, a diagnosis of Rosai-Dorfman disease was established.

Randomized controlled trial comparing cosmetic results of midline incision versus off-midline incision for specimen extraction in laparoscopic colectomy.

PURPOSE: A notable advantage of laparoscopic colorectal surgery is that only a small incision at the extraction site is necessary, which is considered to be cosmetically beneficial. Meanwhile, the optimal extraction site for the resected specimen in laparoscopic colectomy is controversial in terms of cosmetic benefit. This randomized controlled trial compares midline and off-midline extraction sites in laparoscopic colectomy in patients with colon cancer, with consideration of cosmetic benefits as the primary endpoint. METHODS: Included were patients that underwent elective laparoscopic colectomy at WMUH between October 2014 and February 2017. Patients were randomly assigned to either midline incision group or off-midline incision group. Prospectively collected data included cosmetic results (patients and observer assessment scale) and complications including incidence of incisional hernia, SSI, and pain. This trial was registered with UMIN Clinical Trials (UMIN000028943). RESULTS: Finally, 98 patients with colorectal cancer were analyzed. No significant differences were found between the two groups in patient and observer assessment scales of cosmetic results (midline 8 ± 1.1 vs off-midline 11 ± 5.9 p = 0.16, midline 13.5 ± 6.6 vs off-midline 15 ± 11 p = 0.58, respectively) or in postoperative pain. However, incisional hernia occurred in four cases in the midline group (8%), which was significantly higher than that in the off-midline group (no cases, 0%). CONCLUSION: There was no significant difference in terms of cosmetic benefit, the primary endpoint, between the two groups. In this study, only the extraction site location was compared; future studies will examine differences depending on the incisional direction, including the incidence of incisional hernia.

Risk factors of first bite syndrome after surgical resection of parapharyngeal space tumors.

OBJECTIVE: First bite syndrome is a complication of surgical resection of parapharyngeal space tumors and the development of cramping pain in the parotid region with the first bite of a meal. The present study aimed to identify the potential risk factors for the development of first bite syndrome. METHODS: We retrospectively reviewed 30 consecutive patients with parapharyngeal space tumors who had been surgically treated between August 2003 and December 2015 at our department. RESULTS: The tumor site (prestyloid or retrostyloid) and surgical approach (transcervical-parotid, transparotid, or transcervical) were not correlated with the development of first bite syndrome. Ligation and mobilization of the external carotid artery was significantly correlated with the development of first bite syndrome. Moreover, patients with complete resection of the parotid gland did not experience first bite syndrome. DISCUSSION: The present findings suggest that concomitant surgical settings of 1) sympathetic denervation of the parotid gland with ligation of the external carotid artery or injury of the sympathetic nerve plexus around the external carotid artery during its mobilization, and 2) residual parotid gland tissue are risk factors for the development of first bite syndrome after surgical resection of parapharyngeal space tumors. J. Med. Invest. 70 : 150-153, February, 2023.

Elective colorectal cancer surgery in nonagenarians and postoperative outcomes.

OBJECTIVES: The population of very elderly patients is increasing, and nonagenarians have significantly higher mortality rates and poorer rates of survival than younger patients. Meanwhile, recent studies have shown colorectal cancer surgery in nonagenarian patients to be feasible regarding postoperative outcomes. This retrospective study aims to evaluate the postoperative outcomes of nonagenarians in the latest clinical settings. METHODS: Consecutive nonagenarian patients who underwent elective colorectal cancer surgery between 2018 and 2020 retrospectively enrolled (Trial registration number: UMIN000046296 on December 7th, 2021). Clinicopathological data and short-term postoperative outcomes were collected for statistical analysis. RESULTS: This study included 81 nonagenarian patients (31 males, 50 females). Postoperative complications occurred in 21 patients (25.9%), and 3 patients died within 90 days (3.7%). Multivariate analysis revealed prognostic nutritional index was a significant predictor of postoperative complications (OR 2.99, 95% CI 0.78-9.10, P = 0.048), and performance status ≥ 3 could be an independent risk factor of 90-day mortality (HR 32.30, 95% CI 3.20-326.10, P = 0.032). CONCLUSIONS: Short-term outcomes after surgical treatment for nonagenarian patients with colorectal cancer were acceptable. Low prognostic nutritional index was closely related to postoperative complications and poor performance status could also lead to 90-day mortality. In aging populations, risk stratification to prevent poorer postoperative outcomes in nonagenarian patients is needed.

New diagnostic strategy using narrow-band imaging (NBI) during laparoscopic surgery for patients with colorectal cancer.

BACKGROUND: Accurate tumor stage diagnosis during laparoscopic surgery remains difficult. We clarify the impact of new diagnostic strategy using narrow-band imaging (NBI) during laparoscopic surgery for colorectal cancer compared with other strategies. METHODS: We defined angiogenesis (Ag) and fibrosis (Fib) grades using NBI laparoscopy (lap-NBI), and assessed the clinicopathological features associated with these grades for 67 patients with colorectal cancer who underwent surgery. We assessed vessel density and gray scale with computer software. RESULTS: NBI-Ag-grade and NBI-Fib-grade of the serosal surface of cancer lesions and peritoneal nodules correlated with vessel density and gray scale of those assessed by Image J computer software. NBI-Fib-grades of liver nodules also correlated with gray scale. NBI-Ag- grade and Fib-grade of the serosal surface of cancer lesions correlated with pathological depth of invasion. These NBI grades of pathological metastatic peritoneal nodules were higher than those of pathologically benign peritoneal nodules. NBI- Fib grades of pathological metastatic liver nodules were higher than those of pathologically benign liver nodules. In multivariate analysis, lap-NBI was associated with different diagnosis for T3, T4 and non-T3, and non-T4. Moreover, lap-NBI was associated with different diagnosis for T4 and non-T4. Predictive value for T4 by lap-NBI showed high sensitivity (85%) specificity (87%), positive predictive value (74%), negative predictive value (93%), and overall accuracy (87%). Sensitivity and overall accuracy of lap-NBI was superior to that of other diagnostic modalities. CONCLUSION: We clarified the usefulness of the new diagnostic strategy using lap-NBI during laparoscopic surgery for colorectal cancer in comparison with other strategies.

What is related to postoperative outcome of frail status in elective colorectal cancer surgery?

Background: The population affected by colorectal cancer is growing, and there is an increasing need for prevention of functional decline following treatment. We proposed that the Kihon Checklist published by the Japanese Ministry of Health, Labor, and Welfare would be an appropriate means of frailty assessment for prediction of postoperative complications in older patients with colorectal cancer. This prospective cohort study aims to identify the factors influencing postoperative frailty. Methods: We prospectively enrolled consecutive patients with colorectal cancer and aged ≥ 65 year (N = 500) between May 2017 and December 2018. Eligible patients were assessed with the Kihon Checklist prior to surgery and 30 days after surgery. The main measures were variables related to postoperative change in view of frail status. Results: According to the Kihon Checklist questionnaire, 164 patients were frail preoperatively and 172 patients were frail postoperatively, whereas 38 patients changed from "nonfrail" before surgery to postoperative "frail." Overall complications were counted in 97 patients (19.4%), and 5 patients died. Performance status ≥ 2, history of laparotomy, open surgery, complication, ostomy creation, and delirium were significantly associated with changing postoperative "frail" (P = .014, P = .023, P = .006, P < .001, P = .023, and P = .024, respectively). In multivariate analysis, independent related factors of changing postoperative "frail" were complication (odds ratio 2.69, 95% confidence interval 1.19-6.09, P = .018) and ostomy creation (odds ratio 2.32, 95% confidence interval 1.01-5.33, P = .047). Conclusion: The Kihon Checklist questionnaire could identify the factors related to postoperative change of frailty status in older patients with colorectal cancer. This cohort concluded that whether postoperative complication occurred or not was closely associated with perioperative change of frailty status.

Successful endoscopic diagnosis of angiosarcoma of the small intestine: A case report.

A 51-year-old man had hematochezia, anemia, and an intraabdominal mass. Gastroscopy and colonoscopy showed no significant lesions with intraluminal bleeding, while radiological examinations showed bulky swelling of the lymph nodes around the abdominal aorta and a tumor in the left ischial bone. Small intestine endoscopy detected a dark purpuric protruding tumor of the jejunum and its biopsy specimen brought a definitive diagnosis of primary jejunal epithelioid angiosarcoma from positive staining of AE1/AE3, CD31, and erythroblast transformation specific related gene in immunohistochemical studies. The patient underwent surgical resection with adjuvant chemotherapy but died of progression of metastases 7 months after the diagnosis. Epithelioid angiosarcoma of the gastrointestinal tract is an extremely rare malignancy with poor prognosis and it is challenging to distinguish from undifferentiated carcinoma or melanoma. Immunohistochemistry is necessary for a definitive diagnosis. Sufficient biopsy specimen may aid a prompt diagnosis of this disease of the small intestine, which may present as obscure gastrointestinal bleeding.

[Disseminated cryptococcosis during ibrutinib treatment for chronic lymphocytic leukemia].

We report a case of invasive fungal infection (IFI) that ensued during ibrutinib treatment. A 79-year-old female was diagnosed with chronic lymphocytic leukemia seven years prior. She had undergone chemotherapy at the ages of 72 and 75. Subsequently, she was placed on ibrutinib treatment at the age of 79. On the 119th day after the ibrutinib treatment initiation, she was admitted to our hospital with the complaints of frequent urination and hematuria, and three days later, she died of disseminated cryptococcosis.IFIs should be considered in the event of infections that develop early after the ibrutinib treatment initiation.

Laparoscopic anterior resection with or without transanal tube for rectal cancer patients - A multicenter randomized controlled trial.

BACKGROUND: In rectal cancer surgery, insertion of transanal tube has been shown to have efficacy to prevent anastomotic leakage. This randomized controlled study aims to clarify the incidence of anastomotic leakage with or without transanal tube in patients with rectal cancer. METHODS: Patients who underwent elective low anterior resection were randomly allocated to either have transanal tube insertion or not for five days after surgery. We examined the incidence of anastomotic leakage, postoperative 30-day morbidity and mortality. RESULTS: 157 patients were randomized to the transanal tube group or the no-transanal tube group. Symptomatic anastomotic leakage occurred in six patients (7.6%) of the former group and eight patients (10.3%) in the latter group, without significant difference (p = 0.559). There was also no significant difference in morbidity between groups (p = 0.633) and no mortality was detected. CONCLUSIONS: Transanal tube insertion had no significant benefit towards prevention of anastomotic leakage in rectal cancer surgery.

Preoperative detection of KRAS mutated circulating tumor DNA is an independent risk factor for recurrence in colorectal cancer.

Preoperative ctDNA status in relation to recurrence in cases of CRC remains unclear. We examined preoperative ctDNA detection by targeting KRAS gene mutations as a predictive marker for recurrence after CRC surgery. We measured the preoperative KRAS mutated ctDNA status and analyzed the correlation with clinicopathologic features of 180 patients that underwent surgery for CRC. We studied the association between preoperative KRAS mutated ctDNA and postoperative recurrence in patients (n = 150) that underwent radical surgery. KRAS mutated ctDNA was detected in 59 patients (32.8%). Median mutant allele frequency of KRAS in ctDNA was 0.20%. KRAS status in ctDNA and lymph node metastasis and distant metastasis were not significantly different. Among patients that underwent radical resection, recurrence occurred in 21 (14.0%, median follow-up 24 months). In Kaplan-Meier analysis, preoperative detection of KRAS mutated ctDNA was associated with inferior recurrence-free interval (RFI) (p = 0.002) and recurrence-free survival (RFS) (p = 0.025). In a multivariate Cox proportional hazards model, preoperative detection of KRAS mutated ctDNA was an independent factor related to both RFI (HR = 3.08; p = 0.012) and RFS (HR = 2.18; p = 0.044). Preoperative measurement of KRAS mutated ctDNA could be useful to decide postoperative treatment.

Optimal Assessment of Frailty Predicts Postoperative Complications in Older Patients with Colorectal Cancer Surgery.

OBJECTIVES: The increasingly elderly worldwide population has affected the incidence of colorectal cancer. Establishment of reliable assessment of frailty and proposals for multi-disciplinary interventions are urgently required in oncology practices. Kihon Checklist (KCL) was published by the Japanese Ministry of Health, Labor and Welfare originally to identify individuals ≥ 65 years old at probable risk for requiring care or social support. We investigate the validity of KCL for frailty assessment to predict postoperative complication in older patients with colorectal cancer. METHODS: Consecutive colorectal cancer patients aged ≥ 65 (n = 500) were prospectively examined between May 2017 and December 2018. Preoperative frailty assessment was conducted by the G8 questionnaire and KCL. The main outcome measures were correlation between frailty, other clinical variables, and postoperative complications within 30 days after elective surgery. RESULTS: Of the 500 patients, 278 (55.6%) and 164 (32.8%) patients were classified as 'frail' by G8 and KCL, respectively. Overall complications counted among 97 patients (19.4%), and they were significantly associated with KCL ≥ 8-frail (46/164, p = 0.001), as opposed to G8 ≤ 14-frail (56/278, p = 0.531). Multivariate analysis showed that KCL ≥ 8 (hazard ratio 1.88, 95% confidence interval 1.16-3.04, p = 0.011) was an independent risk factor for these complications. CONCLUSIONS: KCL assessment can identify frail older patients likely to suffer from postoperative complications after colorectal cancer surgery. Preoperative screening of frailty, particularly by KCL, would help older patients prevent their worse outcomes in colorectal cancer. TRIAL REGISTRATION: UMIN000026689.

Small intestinal arteriovenous malformation treated by laparoscopic surgery using intravenous injection of ICG: Case report with literature review.

INTRODUCTION: Approximately 5 % of gastrointestinal bleeding is due to small intestinal bleeding. Bleeding from small intestinal arteriovenous malformation (AVM) is rare, with few reported cases. Finding the precise location and boundary is difficult during surgery, so we tried using intravenous injection of indocyanine green (ICG). Use of ICG in a case of intestinal AVM is reported here for the first time, with a review of the literature. PRESENTATION OF CASE: A 48-YEAR-old male had anemia and low hemoglobin level (Hb) 4.0 g/dL. After several examinations including small intestinal endoscopy, capsule endoscopy and angiography, AVM was identified. Preoperative diagnosis was AVM caused by branching of the ileocolic artery (ICA). Meanwhile, macroscopy showed engorgement of the vein in the ileum wall and mesentery, the boundary of which was unclear. We performed intra-operative monitoring with ICG. After intravenous injection of ICG, the boundary and location became clear. The abnormal ileum was 30 cm in length and located 130 cm from the Treitz ligament, which was different from angiographic findings. Pathology showed dilated vascular hyperplasia of the submucosa, tunica and chorionic membrane. Final diagnosis was ileum AVM. The postoperative course was uneventful and gastrointestinal bleeding stopped. CONCLUSIONS: ICG monitoring aided diagnosis and treatment of Ileum AVM, which was treated by laparoscopic surgery.

Importance of pleural findings in patients with amyloid cardiomyopathy complicated with refractory pleural effusion.

A 76-year-old male was admitted to our hospital for progressive bilateral pleural effusion. Because of typical echocardiographic findings such as left ventricular (LV) hypertrophy, thickness of the mitral valve, and a granular sparkling appearance of the LV wall, amyloid cardiomyopathy was suspected. Regardless of up-titration of several diuretic agents, the bilateral pleural effusion did not improve. Because the histological findings of the right ventricular septum (direct-fast-scarlet staining) obtained by biopsy that demonstrated amyloid deposits in perivascular and pericellular lesions, amyloid cardiomyopathy was diagnosed. However, cardiac catheterization revealed normal right and left atrial pressure and normal right and left ventricular end-diastolic pressure. Therefore, hemodynamic deterioration was less likely to be the cause of persistent pleural effusion. Amyloid deposits were also detected in the pleural biopsy specimen, so pleural amyloidosis was diagnosed and may have played an important role in the refractoriness of the pleural effusion. .

Quantitative Indocyanine Green Fluorescence Imaging Used to Predict Anastomotic Leakage Focused on Rectal Stump During Laparoscopic Anterior Resection.

Background: Anastomotic leakage (AL) is arguably the most troublesome complication of anterior resection (AR). In recent years, however, indocyanine green (ICG) fluorescence imaging has been recently used to evaluate blood flow in the anastomosis site, and it has been suggested that AL may be predicted. We reported the effectiveness of predicting AL in colorectal cancer surgery by observing a quantitative laparoscopic ICG fluorescence imaging for the first time. The purpose of this study was to predict the risk of postoperative AL by quantitative laparoscopic ICG fluorescence imaging focused on the rectal stamp, which is one of the major causes of AL in AR, and to construct diverting stoma (DS) only in appropriate cases. Methods: We studied the 25 patients who underwent elective laparoscopic AR for rectal cancer at our hospital between July 2016 and June 2017. Before enforcing double-stapling technique anastomosis, we injected ICG intravenously, and laparoscopically evaluated blood flow on the rectal stump. We analyzed quantitatively the relationship between various parameters and AL. Results: Median T0, from when the ICG was injected intravenously and the ICG disappeared from the injection route to the rise of the histogram of intensity, in AL group was significantly longer than that in non-AL group (P = .03). There were no other significant differences between AL and non-AL groups. Conclusions: T0 was longer in patients with AL than in those without. If prolonged T0 can be recognized intraoperatively, it will be possible to construct DS for appropriate patients only.

Modulation of capecitabine administration to improve continuity of adjuvant chemotherapy for patients with colorectal cancer: A phase II study.

Adjuvant chemotherapy with capecitabine is frequently not completed due to adverse events, including hand-foot syndrome. A higher completion rate of capecitabine by reduction of the side effects may improve disease-free survival and quality of life of affected patients. In the present study, colorectal cancer (CRC) patients were treated with capecitabine (2,500 mg/m2/day), which was taken for five days, followed by an interval of two days (5-days-on/2-days-off schedule). One course lasted three weeks, and eight courses (24 weeks) were administered. The median number of treatment courses was significantly higher in patients in the 5-days-on/2-days-off regimen group compared with that of patients in the retrospectively included conventional regimen group (P=0.0438). The frequency of completion of the scheduled treatment by patients in the 5-days-on/2-days-off regimen group was significantly higher (P=0.0389). The present phase II study suggests that toxicities associated with the 5-days-on/2-days-off regimen are lower compared with those of the conventional regimen, and that the occurrence of adverse events was higher, but less high-grade toxicities were reported. The time to treatment failure was also favorable in the new regimen and it demonstrated good feasibility. In conclusion, the present study demonstrated good feasibility with retained quality of life and acceptable adverse effects (mostly low-grade), and the 5-days-on/2-days-off regimen should be further evaluated in future randomized controlled trials. The present study was registered in the University Hospital Medical Information Network (UMIN) clinical trial registry (no. UMIN000012813).

Corticobasal degeneration with deep white matter lesion diagnosed by brain biopsy.

Corticobasal degeneration (CBD) is a rare progressive neurodegenerative disorder characterized by asymmetric presentation of cerebral cortex signs, cortical sensory disturbance and extrapyramidal signs. Herein, we report a case of a 66-year-old Japanese woman who presented with apraxia of the right hand. She subsequently developed postural instability and cognitive impairments that rapidly worsened. One and a half years later, the patient was wheelchair-bound and severely demented. Brain magnetic resonance imaging revealed left dominant atrophy of the frontoparietal lobe. There was a hyperintense lesion in the deep white matter expanding toward the subcortical area on fluid-attenuated inversion recovery (FLAIR) images. In order to rule out the possibility of an intracranial tumor such as an astrocytoma or malignant lymphoma, we performed a brain biopsy of the left frontal middle gyrus. The patient became bedridden and showed akinetic mutism 1 year after biopsy. Pathological examination revealed a large amount of 4-repeat tau-immunoreactive neuropil threads scattered predominantly in the corticomedullary junction and tau-immunoreactive structures, consistent with CBD. Immunostaining for p53 showed no positive cells, and there were very few Ki-67-positive cells. On immunoblots of sarkosyl-insoluble brain extracts, a major doublet of 64 and 68 kDa full-length tau with two closely related fragments of approximately 37 kDa were detected. Based on these results, the patient was pathologically diagnosed as having CBD, excluding the possibility of tumor. Taken together with previous similar case reports, our findings indicate that a deep white matter hyperintense lesion on FLAIR images may be a useful clue to CBD, predicting rapid clinical progression with severe dementia based on severe white matter degeneration with a large amount of tau accumulation on pathological examination.

Tumor Sidedness Is Associated with Survival in Patients with Synchronous Colorectal Peritoneal Carcinomatosis.

BACKGROUND: Synchronous colorectal peritoneal carcinomatosis (SCPC) is not a rare entity, and the prognosis is extremely poor compared to other metastatic sites such as the liver and lung. Therefore, optimal treatment based on tumor characteristics is needed. Recently, the tumor sidedness of colorectal cancer has been reported as one of the prognostic factors and also as a key factor for the treatment strategy. The purpose of this study was to assess the clinical impact of tumor sidedness in patients with SCPC. METHODS: A total of 189 cases of SCPC were identified in a retrospective database at Wakayama Medical University Hospital (WMUH) between 1998 and 2014, and were analyzed with a special focus on tumor location. RESULTS: In multivariate analysis, a right-sided location (p = 0.02) and the presence of liver metastases (p < 0.001) were found to be the worst prognostic factors. The median survival time (MST) with right-sided and with left-sided SCPC was 10 and 16 months, respectively. The right-sided SCPC group included more aged patients (p = 0.045) and fewer patients who received postoperative chemotherapy (p = 0.034). When we focused on patients with macroscopically complete resection (n = 39), the MST and disease-free survival in the right-sided SCPC group was significantly shorter than in the left-sided SCPC group (p = 0.030 and p = 0.043, respectively). The MST of the right-sided and the left-sided SCPC patients among the completely resected patients was 24 and 73 months, respectively. CONCLUSION: Tumor sidedness may be a potent prognostic indicator for patients with SCPC. The survival time with right-sided SCPC is dramatically reduced compared to that with left-sided SCPC, especially among completely resected cases. We should change the treatment strategy according to the location of SCPC.

Short-term pre-operative systemic administration with low-dose of steroid does not make a false-negative diagnosis of definite eosinophilic chronic rhinosinusitis after endoscopic sinus surgery.

According to JESREC criteria, definite eosinophilic chronic rhinosinusitis (ECRS) is postoperatively diagnosed based on over 70 eosinophils in high power magnification fields of the resected nasal polyps in patients with probable ECRS. Preoperative systemic administration with steroid is a standard practice, because it reduced intraoperative bleeding during endoscopic sinus surgery (ESS) in patients with ECRS. However, it was recently reported that systemic administration with steroid decreased the number of eosinophils in the nasal polyps, leading to a false negative diagnosis of definite ECRS. To overcome the risk, we have adopted short-term pre-operative systemic administration with low-dose of steroid and examined if our steroid administration makes a false-negative diagnosis. We performed a retrospective chart review of 42 patients with probable ECRS. Eleven patients were administered with 0.5 mg of bethametasone for 7 days before ESS, and other 31 patients were not administered. The average number of eosinophils in nasal polyps in patients who were administered with steroid was 188 ± 167, which was not different from 199 ± 149 in the patients who were not administered. These findings suggest that short-term preoperative administration with low-dose of steroid has few risk of a false-negative diagnosis of definite ECRS. J. Med. Invest. 66 : 233-236, August, 2019.

Brain metastasis from cutaneous squamous cell carcinoma coexistent with extramammary Paget's disease: A case report.

We report a case of cutaneous squamous cell carcinoma (CSCC), initially coexisting with invasive extramammary Paget's disease (EMPD), in the scrotum of an 84-year-old man. The patient initially had a rash and pruritus before presenting with a pedunculated scrotal mass surrounded by widespread erythema. He underwent total gastrectomy for adenocarcinoma 1 year previously and had been receiving TS-1 (Tegafur/Gimeracil/Oteracil pottasium) orally. Histopathologically, the tumor consisted of invasive SCC, with invasive EMPD in the erythematous region. From the clinical presentation and histopathological findings, we assumed that CSCC developed in the background of the EMPD. The CSCC metastasized to several inguinal lymph nodes and to the brain in the following years. While the histogenesis of each of the tumors remains to be elucidated, the fact that the CSCC rather than the EMPD metastasized to a distant site in this patient is to be noted for future treatment considerations.