BACKGROUND: Lentigo maligna melanoma (LMM) is a subtype of melanoma that typically develops on sun-damaged skin. LMM is estimated to comprise 4~15% of melanomas, but the prevalence is known to be relatively lower in the Korean population than in the Caucasian population. OBJECTIVE: To review the clinico-pathologic features and treatment outcomes of Korean patients with LMM. METHODS: Nineteen patients diagnosed with LMM during 2003~2015, in the Yonsei University Health System, were included in this study. The age and sex of the patients, lesion location, thickness (Breslow), stage, treatment methods, BRAF, NRAS, and KIT mutation status, and survival rates were analyzed. RESULTS: Among the 19 Korean patients, 11 were male and 8 were female. The median age was 59.2 years. The most common site was the cheek (47.4%), followed by the scalp, eyelid, nose, forehead, lip, and neck. At the time of diagnosis, 13 patients were in localized stages (5 patients, stage 0; 3 patients, stage I; and 5 patients, stage II) and 6 patients were in advanced stages (3 patients, stage III; and 3 patients, stage IV). Patients in the localized stages showed better overall survival (OS) than those in the advanced stages (p=0.012). Nine patients were treated with a wide excision, and 6 using Mohs micrographic surgery. Three patients received high-dose interferon-α therapy; 6, chemotherapy; and 4, radiotherapy. Two patients in stage 0 were treated with topical ingenol mebutate. Two patients had BRAF V600E mutation; 1, NRAS G12R mutation; and 1, KIT mutation. Median OS of the patients was 40.8 months. CONCLUSION: Our analysis provides additional information about clinical characteristics, treatment, and prognosis of LMM in Korean patients.
Cheek ; Diagnosis ; Drug Therapy ; Eyelids ; Female ; Forehead ; Humans ; Hutchinson's Melanotic Freckle* ; Lentigo* ; Lip ; Male ; Melanoma* ; Mohs Surgery ; Neck ; Nose ; Prevalence ; Prognosis ; Radiotherapy ; Retrospective Studies* ; Scalp ; Skin ; Survival Rate

Adolescent ; Carcinoma, Renal Cell ; metabolism ; pathology ; Desmin ; metabolism ; Diagnosis, Differential ; Humans ; Leg ; MART-1 Antigen ; metabolism ; Male ; Melanoma ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Sarcoma, Clear Cell ; metabolism ; pathology ; Skin Neoplasms ; metabolism ; pathology ; surgery

OBJECTIVE: To analyze and summarize clinical characteristic, treatment scheme and survival rate of primary melanom in nasal cavity and sinus. METHOD: We retrospectively analyzed the 9 patients with primary melanom in nasal cavity and sinus who in data proceed were treated and reviewed the related literature. RESULT: Among the 9 patients, the clinical main symptoms are rhinostegnosis of lateral nasal and intermittent nasal bleeding. Pathologic examination is mainly characterized by tumor cells abnormity and cytoplasm containing pigment or without pigment, and main diagnosis basis is some or all of the positive for HMB45, S-100, melan-A. The survival rate are 88.9% (8/9) of 1-year, 66.7% (6/9) of 3-year and 33.3% (3/9) of 5-year. CONCLUSION The incidence of primary melanom in nasal cavity and sinusis is not frequent in clinic and confirmed by immunohistochemical. The extensive radical excision of focus and combine adjuvant radiotherapy postoperative may improve the survival rate of patients.
Aged ; Aged, 80 and over ; Female ; Follow-Up Studies ; Humans ; Male ; Melanoma ; diagnosis ; surgery ; Middle Aged ; Nasal Cavity ; Nose Neoplasms ; diagnosis ; surgery ; Paranasal Sinus Neoplasms ; diagnosis ; surgery ; Retrospective Studies

OBJECTIVETo study the clinicopathologic features and prognosis of plasmacytoid urothelial carcinoma (PUC) of the urinary bladder.

METHODSThe clinical and pathologic findings of 16 cases of PUC were retrospectively reviewed. Immunohistochemical study (MaxVision method) was carried out. The follow-up data were analyzed.

RESULTSThere were altogether 15 males and 1 female. The age of patients ranged from 40 years to 85 years (median = 64 years). Most patients (15/16) presented with hematuria. The tumor cells were small to medium in size and contained eccentric nuclei and moderate to abundant eosinophilic cytoplasm, assuming a plasmacytoid appearance. The architectural pattern varied from loosely cohesive sheets to cords, papillae, small nests or gland-like structures. Most tumors invaded into the lamina propria or muscularis propria. Twelve of the 16 cases had concurrent conventional urothelial carcinoma component. Immunohistochemical study showed that the tumor cells in all cases were strongly positive for AE1/AE3, epithelial membrane antigen, CK7 and CK18. CK20 and uroplakin III were also expressed in 9 cases. CEA, p53, CD138, p63 and E-cadherin were positive in 12, 13, 15, 11 and 10 cases, respectively. Ki-67 index ranged from 5% to 70% (mean = 30%). All tumors were negative for vimentin, LCA, kappa/lambda light chains, S-100 protein, HMB 45,Melan A, smooth muscle actin and desmin. Follow-up information was available in 13 patients. The duration of follow up ranged from 3 months to 10 years. Three patients died of distant metastasis at 3, 27 and 60 months after the operation, respectively. One patient was alive with disease at 25 months. One was alive at 43 months with a prior recurrence. Another 8 patients were alive and disease free at 7 to 120 months.

CONCLUSIONSPUC of the urinary bladder is a rare variant of high-grade urothelial carcinoma. Immunohistochemical study with positivity for CK7, CK20, p63 and uroplakin III and negative staining for vimentin and LCA may be helpful in the differential diagnosis. PUC is a malignant tumor with high invasiveness, high recurrence rate and poor prognosis. Radical cystectomy is considered as the first line treatment for PUC.

Adult ; Aged ; Aged, 80 and over ; Biomarkers, Tumor ; metabolism ; Carcinoma, Signet Ring Cell ; metabolism ; pathology ; Carcinoma, Transitional Cell ; metabolism ; pathology ; surgery ; Cystectomy ; methods ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Keratin-20 ; metabolism ; Keratin-7 ; metabolism ; Male ; Melanoma ; metabolism ; pathology ; Membrane Proteins ; metabolism ; Middle Aged ; Neoplasm Recurrence, Local ; Plasma Cells ; pathology ; Plasmacytoma ; metabolism ; pathology ; Prognosis ; Retrospective Studies ; Syndecan-1 ; metabolism ; Urinary Bladder Neoplasms ; metabolism ; pathology ; surgery ; Uroplakin III ; metabolism

Actins ; metabolism ; Adult ; Diagnosis, Differential ; Endometrial Neoplasms ; metabolism ; pathology ; surgery ; Endometrial Stromal Tumors ; metabolism ; pathology ; surgery ; Female ; Humans ; Hysterectomy ; Leiomyoma, Epithelioid ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Perivascular Epithelioid Cell Neoplasms ; metabolism ; pathology ; surgery ; Receptors, Progesterone ; metabolism ; Sarcoma, Clear Cell ; metabolism ; pathology ; Uterine Neoplasms ; metabolism ; pathology ; surgery

Actins ; metabolism ; Angiomyoma ; metabolism ; pathology ; surgery ; Cranial Fossa, Middle ; Diagnosis, Differential ; Follow-Up Studies ; Humans ; Magnetic Resonance Imaging ; Male ; Melanoma-Specific Antigens ; metabolism ; Middle Aged ; Perivascular Epithelioid Cell Neoplasms ; immunology ; S100 Proteins ; metabolism ; Skull Base Neoplasms ; metabolism ; pathology ; surgery

BACKGROUND: A retrospective study was conducted to review the overall survival and treatment outcomes of high grade melanoma in the extremity to explore the clinical features of malignant melanoma of the hand and foot, and the therapeutic efficacies and survival rate after amputation. METHODS: The clinical data of 30 patients with malignant melanoma of the hand and foot (confirmed by pathological examination), who were admitted and treated in our hospital between 2001 and 2010, were analyzed retrospectively. We analyzed variables affecting overall and disease-free survival. RESULTS: Thirty patients (18 men and 12 women) treated with an amputation procedure for malignant melanoma in the hand or foot constituted the study cohort. The average age of the patients at the time of diagnosis was 58.7 years. Univariate analysis for overall melanoma survival revealed that diagnosis at over 70 years of age, postoperative lymph node metastasis, and location of the tumor were significant prognostic factors. And on the Kaplan-Meier survival curve, old age, American Joint Committee on Cancer stage and postoperative lymph node metastasis showed statistically significant differences in the 5-year survival rate. Also, amputation with aggressive lymph node dissection showed improved long term survival in advanced stage melanoma. CONCLUSIONS: In Korean melanoma patients, for the treatment of high grade melanomas in the extremities after amputation, early diagnosis and postoperative follow-up for evaluation of lymph node metastasis are critical factors for long-term survival. And by performing lymph node dissection during amputation, we may improve the survival rate in advanced stage melanoma patients.
Aged ; *Amputation ; Analysis of Variance ; Disease-Free Survival ; Female ; Foot/*surgery ; Hand/*surgery ; Humans ; Kaplan-Meier Estimate ; Male ; Melanoma/diagnosis/pathology/*surgery ; Middle Aged ; Neoplasm Staging ; Prognosis ; Republic of Korea ; Retrospective Studies ; Skin Neoplasms/diagnosis/pathology/*surgery ; Survival Rate ; Treatment Outcome

OBJECTIVETo investigate the clinical pathological features, diagnosis and differential diagnosis of pigmented dermatofibrosarcoma protuberance (PDFSP).

METHODSThe clinical history, histopathological features, immunohistochemical characteristics, treatment and prognosis were analyzed in seven cases of PDFSP. Fluorescence in situ hybridization (FISH) was used to detect the expression of COL1A1/PDGFB fusion gene, and related literature was reviewed.

RESULTSThe median age of the seven patients (4 females, 3 males) was 47 years with the tumors involving mostly the trunk (four cases). Histologically, PDFSP showed a cellular lesion composed of spindle cells arranged in short fascicles that form a distinct storiform pattern, and the pigmented bipolar or multipolar dendritic cells were present with tentacle like processes emanating from a nucleus containing zone. One case showed fibrosarcomatous change. The pigment was tinctorially similar to melanin. The spindle cells were positive for CD34 and vimentin, but negative for HMB45, Melan A, S-100, desmin, CD68 or α-SMA. HMB45, Melan A, S-100 and vimentin were expressed in the melanin containing cells in 4, 4, 5 and 7 cases, respectively. The labeling index of Ki-67 was 1%-8%. Among the 4 cases successfully examined by FISH, 3 showed t(17;22)(q21;q13) which suggested COL1A1/PDGFB fusion gene. Three patients were treated by wide local excision and four were treated by simple surgical excision. Two patients developed recurrences during the follow-up period of 12 to 123 months. Of those treated by wide local excision, none developed recurrence. No patient died in the follow-up period.

CONCLUSIONSPDFSP is a rare pigmented variant of DFSP and an intermediate grade malignant tumor. The orgin of the tumor cells is still controversial. Surgical pathologists and dermatopathologists need to be aware of the prototypical histological appearance of PDFSP as there is a risk of misdiagonsing it as either pigmented tumors associated with neurocutaneous syndromes or a highly malignant melanocytic neoplasm.

Adult ; Aged ; Antigens, CD34 ; metabolism ; Child, Preschool ; Dermatofibrosarcoma ; diagnosis ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; In Situ Hybridization, Fluorescence ; MART-1 Antigen ; metabolism ; Male ; Melanoma ; metabolism ; pathology ; Melanoma-Specific Antigens ; metabolism ; Middle Aged ; Neoplasm Recurrence, Local ; Neurilemmoma ; metabolism ; pathology ; Neurofibroma ; metabolism ; pathology ; Oncogene Proteins, Fusion ; metabolism ; Prognosis ; Retrospective Studies ; S100 Proteins ; metabolism ; Skin Neoplasms ; diagnosis ; metabolism ; pathology ; surgery ; Vimentin ; metabolism

Aged ; Carcinoma, Medullary ; metabolism ; pathology ; surgery ; Carcinoma, Squamous Cell ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Gastrectomy ; Humans ; Keratins ; metabolism ; Lymphoma ; metabolism ; pathology ; Melanoma ; metabolism ; pathology ; Middle Aged ; Neoplasm Staging ; RNA, Viral ; metabolism ; Retrospective Studies ; Stomach Neoplasms ; metabolism ; pathology ; surgery

Perivascular epithelioid cell tumor (PEComa) is extremely rare, which originated from mesenchymal cells in the intestine, and composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. We report here on a case of PEComa in the sigmoid colon. A 62-year-old woman presented with hematochzia 10 days ago. Her abdominal computed tomography scan showed a 5 cm sized intraluminal fungating heterogeneously enhanced, high density mass, which infiltrated pericolic tissue surrounding the sigmoid colon. Colonoscopy showed a purple colored polypoid mass with lobulating contour in the sigmoid colon. She underwent laparoscopic anterior resection. On the histologic examination, the tumor consisted of polygonal epithelioid cells with sheet-like growth of nests, which looked like alveolar tissues in lung. The tumor cells were strongly positive stained with human melanoma black-45 (HMB-45). Pathologic examination was compatible with PEComa. Sixteen months after surgery, she did well without tumor recurrence after surgery. We review the literatures concerning PEComa of the intestine focusing on endoscopic findings.
Colonic Neoplasms/diagnosis ; Colonoscopy ; Female ; Gastrointestinal Hemorrhage ; Humans ; Melanoma-Specific Antigens/metabolism ; Middle Aged ; Perivascular Epithelioid Cell Neoplasms/*diagnosis/pathology/surgery ; Tomography, X-Ray Computed