Ilson Sepúlveda, Á. Compan, J. Ulloa, Pablo Mucientes, P. Vera, R. Ascui, Felipe Fredes
Undifferentiated high-grade pleomorphic sarcoma is a tumor of mesenchymal cells without clear cell differentiation and represents a diagnosis of exclusion. It is located mainly in extremities, so its location in the head and neck is extremely rare. We report the case of an undifferentiated high-grade pleomorphic sarcoma located on the hard palate.
{"title":"Maxillary undifferentiated high-grade pleomorphic sarcoma: A case report and review of the literature","authors":"Ilson Sepúlveda, Á. Compan, J. Ulloa, Pablo Mucientes, P. Vera, R. Ascui, Felipe Fredes","doi":"10.5430/CRCP.V4N1P32","DOIUrl":"https://doi.org/10.5430/CRCP.V4N1P32","url":null,"abstract":"Undifferentiated high-grade pleomorphic sarcoma is a tumor of mesenchymal cells without clear cell differentiation and represents a diagnosis of exclusion. It is located mainly in extremities, so its location in the head and neck is extremely rare. We report the case of an undifferentiated high-grade pleomorphic sarcoma located on the hard palate.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"4 1","pages":"32"},"PeriodicalIF":0.0,"publicationDate":"2016-10-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/CRCP.V4N1P32","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210413","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Intradermal nevocelluar nevus is a very common hamartomatous neoplasm of the skin. The author herein reports two tumors of intradermal nevocelluar nevus in the neck occurring in a 55-year-old woman. The tumors were characterized histologically by typical intradermal nevocelluar nevus with significant areas of adenoid or glandular formations. In both tumors, hair follicles were seen in centers of the tumors. The cellular features of the adenoid areas were the same as those of solid areas. In solid areas, vague vacuolated and obvious vacuolated cells were recognized in some areas. In solid areas of the surface and lateral areas of the tumors also showed clear cut adenoid features without vacuolations. The adenoid features showed mild depositions of hemosiderins. There were gradual merges between solid areas and adenoid or glandular areas. Histochemically, the tumor cells including adenoid structures had glycogen, but had no mucins. Immunohistochemically, cells of both tumors were strongly positive for S100 protein, vimentin, NSE, NCAM, and bcl-2, and were weakly positive for synaptophysin and chromogranin. The tumor cells showed low cell proliferative potential; the Ki-67 labeling index was 2%. The tumor cells including adenoid structures were negative for CK AE1/3, CK CAM5.2, desmin, smooth muscle actin, HMB45, p53, p63, CD31, laminin, type 4 collagen, CEA. CD34, CA125, CA19-9, CD45, GFAP, KIT, PDGFRA, CD68, estrogen receptor, progesterone receptor, EMA, MUC1, MUC2, MUC5AC, and MUC6. These histological, histochemical, and immunohistocheimical findings strongly suggested that the present two tumors with adenoid structures belong to intradermal nevocelluar nevus. The formation of the adenoid architectures in the intradermal nevocelluar nevus was discussed. Possible mechanisms appear loss of cell adhesions molecules. The adenoid structures seem not to be degenerative ones. Pathologists should be aware that intradermal nevocelluar nevus may show adenoid structures to avoid diagnostic confusions, for example adenoma and adenocarcinoma of skin appendages. The occurrence of this hitherto undescribed condition in two intradermal nevocelluar nevus in a patient may imply genetic lineage. To elucidate the mechanisms of adenoid structures in intradermal nevocelluar nevus seems future work.
{"title":"Intradermal nevocellular nevus with adenoid features: Report of a case","authors":"T. Terada","doi":"10.5430/CRCP.V4N1P26","DOIUrl":"https://doi.org/10.5430/CRCP.V4N1P26","url":null,"abstract":"Intradermal nevocelluar nevus is a very common hamartomatous neoplasm of the skin. The author herein reports two tumors of intradermal nevocelluar nevus in the neck occurring in a 55-year-old woman. The tumors were characterized histologically by typical intradermal nevocelluar nevus with significant areas of adenoid or glandular formations. In both tumors, hair follicles were seen in centers of the tumors. The cellular features of the adenoid areas were the same as those of solid areas. In solid areas, vague vacuolated and obvious vacuolated cells were recognized in some areas. In solid areas of the surface and lateral areas of the tumors also showed clear cut adenoid features without vacuolations. The adenoid features showed mild depositions of hemosiderins. There were gradual merges between solid areas and adenoid or glandular areas. Histochemically, the tumor cells including adenoid structures had glycogen, but had no mucins. Immunohistochemically, cells of both tumors were strongly positive for S100 protein, vimentin, NSE, NCAM, and bcl-2, and were weakly positive for synaptophysin and chromogranin. The tumor cells showed low cell proliferative potential; the Ki-67 labeling index was 2%. The tumor cells including adenoid structures were negative for CK AE1/3, CK CAM5.2, desmin, smooth muscle actin, HMB45, p53, p63, CD31, laminin, type 4 collagen, CEA. CD34, CA125, CA19-9, CD45, GFAP, KIT, PDGFRA, CD68, estrogen receptor, progesterone receptor, EMA, MUC1, MUC2, MUC5AC, and MUC6. These histological, histochemical, and immunohistocheimical findings strongly suggested that the present two tumors with adenoid structures belong to intradermal nevocelluar nevus. The formation of the adenoid architectures in the intradermal nevocelluar nevus was discussed. Possible mechanisms appear loss of cell adhesions molecules. The adenoid structures seem not to be degenerative ones. Pathologists should be aware that intradermal nevocelluar nevus may show adenoid structures to avoid diagnostic confusions, for example adenoma and adenocarcinoma of skin appendages. The occurrence of this hitherto undescribed condition in two intradermal nevocelluar nevus in a patient may imply genetic lineage. To elucidate the mechanisms of adenoid structures in intradermal nevocelluar nevus seems future work.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"4 1","pages":"26"},"PeriodicalIF":0.0,"publicationDate":"2016-10-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/CRCP.V4N1P26","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210794","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
In 2002, Seibel et al. presented a case series of 18 patients with villous adenomas of the urethra. Since then, only a couple of reports regarding this rare entity have been published. Recently (2013), Kao and Epstein proposed a new entity of tubular adenoma (4 cases) in the urinary tract, being similar to tubular adenomas in gastrointestinal (GI) tract; no following cases have been reported. Herein presented a case of tubule-villous adenoma (TVA) in the prostatic urethra (PU) of a 77-year-old man male patient suffering from dysuria. Cystscopy showed a polypoid/villous tumor of the PU, and transurethral resection of this tumor was performed. Grossly, fragments of the tumor measured 12 mm in diameter and polypoid/villous. Microscopically, it was a TVA indistinguishable from GI adenomas. No urothelium was seen, but a few normal prostatic glands were noted. The tumor showed mild to severe dysplasia, but in situ malignant transformation and invasive malignancy were not seen. Histochemically, acidic mucins were present. Immunohistochemical staining results of the tumor cells were as follows: cytokeratin(CK) AE1/3+++, CKCAM5.2+++, CD34BE12+, CK5+, CK6+, CK7++, CK14-, CK20+, EMA+, CDX-2-, CEA++, CA19-9++, CA125-, vimentin-, NSE-, NCAM-, synaptophysin-, chromogranin-, E-cadherin+++, beta-catenin+++, MUC1++, MUC2-, MUC5AC++, MUC6+, PSA+++, AMACR+++, AFP-, HepPar1-, p53-, KIT-, PDGFRA+, MET+, HER2-, Ki67+ (labeling = 5%). Myoepithelial cells were present in some areas. These results suggest the development and presence of a TVA in the PU indistinguishable from those in the GI tract, and it can arise from prostatic epithelium.
{"title":"Tubulo-villous adenoma with severe dysplasia in the prostatic urethra: A case report and histogenetic considerations","authors":"T. Terada","doi":"10.5430/crcp.v4n1p22","DOIUrl":"https://doi.org/10.5430/crcp.v4n1p22","url":null,"abstract":"In 2002, Seibel et al. presented a case series of 18 patients with villous adenomas of the urethra. Since then, only a couple of reports regarding this rare entity have been published. Recently (2013), Kao and Epstein proposed a new entity of tubular adenoma (4 cases) in the urinary tract, being similar to tubular adenomas in gastrointestinal (GI) tract; no following cases have been reported. Herein presented a case of tubule-villous adenoma (TVA) in the prostatic urethra (PU) of a 77-year-old man male patient suffering from dysuria. Cystscopy showed a polypoid/villous tumor of the PU, and transurethral resection of this tumor was performed. Grossly, fragments of the tumor measured 12 mm in diameter and polypoid/villous. Microscopically, it was a TVA indistinguishable from GI adenomas. No urothelium was seen, but a few normal prostatic glands were noted. The tumor showed mild to severe dysplasia, but in situ malignant transformation and invasive malignancy were not seen. Histochemically, acidic mucins were present. Immunohistochemical staining results of the tumor cells were as follows: cytokeratin(CK) AE1/3+++, CKCAM5.2+++, CD34BE12+, CK5+, CK6+, CK7++, CK14-, CK20+, EMA+, CDX-2-, CEA++, CA19-9++, CA125-, vimentin-, NSE-, NCAM-, synaptophysin-, chromogranin-, E-cadherin+++, beta-catenin+++, MUC1++, MUC2-, MUC5AC++, MUC6+, PSA+++, AMACR+++, AFP-, HepPar1-, p53-, KIT-, PDGFRA+, MET+, HER2-, Ki67+ (labeling = 5%). Myoepithelial cells were present in some areas. These results suggest the development and presence of a TVA in the PU indistinguishable from those in the GI tract, and it can arise from prostatic epithelium.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"4 1","pages":"22"},"PeriodicalIF":0.0,"publicationDate":"2016-10-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/crcp.v4n1p22","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210724","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Appendiceal diverticulosis (AD) is a rare condition. The author reviewed 675 archival pathologic consecutive appendectomies in his institution in the last 12 years. As the results, 6 cases of AD were found. Thus, the incidence of AD was 0.8% of all appendectomies. The AD was seen characteristically in middle aged parsons; in this series, 37-year-old (y) female (F), 42y male (M), 44y F, 56y M, 57y F, and 59y M, with the median of age of 50y, range of 37-59y, and M:F ratio of 3:3. The clinical presentation of the six cases are the same as other appendectomies. The histological features were described.
{"title":"Diverticulosis of vermiform appendix: Incidence and report of 6 cases","authors":"T. Terada","doi":"10.5430/CRCP.V4N1P18","DOIUrl":"https://doi.org/10.5430/CRCP.V4N1P18","url":null,"abstract":"Appendiceal diverticulosis (AD) is a rare condition. The author reviewed 675 archival pathologic consecutive appendectomies in his institution in the last 12 years. As the results, 6 cases of AD were found. Thus, the incidence of AD was 0.8% of all appendectomies. The AD was seen characteristically in middle aged parsons; in this series, 37-year-old (y) female (F), 42y male (M), 44y F, 56y M, 57y F, and 59y M, with the median of age of 50y, range of 37-59y, and M:F ratio of 3:3. The clinical presentation of the six cases are the same as other appendectomies. The histological features were described.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"71 1","pages":"18"},"PeriodicalIF":0.0,"publicationDate":"2016-09-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/CRCP.V4N1P18","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210696","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
M. Campos, P. Varela, A. Baptista, X. Wen, Natividade Rocha
We present a 45-year-old male Caucasian with a one-year history of perianal erythema resistant to topical corticosteroid and antifungal therapy. Physical examination of the perianal region revealed a painful, non-pruriginous erythema with erosions that involved all the circumference of the anus. No oral and no genital lesions were observed. Serologic and virologic markers were collected and a skin biopsy was performed. The markers turned out to be negative and the biopsy revealed the diagnosis of Perianal Paget Disease (PPD). Imunohistochemistry was positive for CK20, EMA and CEA, and negative for CK7. Digital rectal examination suggested a suspicious thickening in the rectum and colonoscopy revealed the presence of a vegetating mass of the distal rectum. Biopsies of the rectal mass displayed a well-differentiated invasive adenocarcinoma. The patient was proposed to neoadjuvant chemo and radiotherapy, followed by abdominoperineal resection with wide surgical excision and lymph node dissection. This case illustrates the importance and difficulties encountered in the clinical diagnosis of this rare disease.
{"title":"Perianal Paget’s Disease with synchronous adenocarcinoma: An unusual diagnosis","authors":"M. Campos, P. Varela, A. Baptista, X. Wen, Natividade Rocha","doi":"10.5430/CRCP.V3N4P66","DOIUrl":"https://doi.org/10.5430/CRCP.V3N4P66","url":null,"abstract":"We present a 45-year-old male Caucasian with a one-year history of perianal erythema resistant to topical corticosteroid and antifungal therapy. Physical examination of the perianal region revealed a painful, non-pruriginous erythema with erosions that involved all the circumference of the anus. No oral and no genital lesions were observed. Serologic and virologic markers were collected and a skin biopsy was performed. The markers turned out to be negative and the biopsy revealed the diagnosis of Perianal Paget Disease (PPD). Imunohistochemistry was positive for CK20, EMA and CEA, and negative for CK7. Digital rectal examination suggested a suspicious thickening in the rectum and colonoscopy revealed the presence of a vegetating mass of the distal rectum. Biopsies of the rectal mass displayed a well-differentiated invasive adenocarcinoma. The patient was proposed to neoadjuvant chemo and radiotherapy, followed by abdominoperineal resection with wide surgical excision and lymph node dissection. This case illustrates the importance and difficulties encountered in the clinical diagnosis of this rare disease.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"3 1","pages":"66"},"PeriodicalIF":0.0,"publicationDate":"2016-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/CRCP.V3N4P66","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210602","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Elmira Vaziri Fard, A. Nael, F. Lin, C. Nangia, B. Wang
Extraskeletal osteosarcoma is a rare soft tissue, high grade malignant mesenchymal neoplasm. It accounts for 1%-2% of all soft tissue sarcomas and 2%-4% of all osteosarcomas. While conventional osteosarcoma of the bone usually occurs in children and adolescents, extraskeletal osteosarcoma is more often seen in mid to late adulthood. The most common sites of this tumor are thigh and buttock. The tumor has a very poor prognosis with a 5-year survival rate of less than 25%. Only 5 cases of primary osteosarcoma have been reported in the parotid gland. We report a case of primary osteosarcoma of the parotid gland in a 68-year-old man. The clinical, radiographic and pathologic features of the tumor are discussed. Despite the very low survival rate of the tumor, our patient was disease free 41 months post surgery.
{"title":"Primary osteosarcoma of the parotid gland: A case report and literature review","authors":"Elmira Vaziri Fard, A. Nael, F. Lin, C. Nangia, B. Wang","doi":"10.5430/CRCP.V3N4P61","DOIUrl":"https://doi.org/10.5430/CRCP.V3N4P61","url":null,"abstract":"Extraskeletal osteosarcoma is a rare soft tissue, high grade malignant mesenchymal neoplasm. It accounts for 1%-2% of all soft tissue sarcomas and 2%-4% of all osteosarcomas. While conventional osteosarcoma of the bone usually occurs in children and adolescents, extraskeletal osteosarcoma is more often seen in mid to late adulthood. The most common sites of this tumor are thigh and buttock. The tumor has a very poor prognosis with a 5-year survival rate of less than 25%. Only 5 cases of primary osteosarcoma have been reported in the parotid gland. We report a case of primary osteosarcoma of the parotid gland in a 68-year-old man. The clinical, radiographic and pathologic features of the tumor are discussed. Despite the very low survival rate of the tumor, our patient was disease free 41 months post surgery.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"3 1","pages":"61"},"PeriodicalIF":0.0,"publicationDate":"2016-09-07","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/CRCP.V3N4P61","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210437","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Background: Trichilemmal carcinoma (TLC) is a rare adnexal malignant tumor developing from the outer root sheath of hair follicles with no distinct clinical features which might clinically be misdiagnosed as actinic keratosis, nodular melanoma, basal or squamous cell carcinoma. Albeit no consensus exists on standard treatment of TLC, tumor excision with adequate clear margins is the current widely accepted treatment consideration with no previous literature on use of radiotherapy (RT) in definitive or postoperative settings. Case presentation: A 60-year-old woman who was repeatedly treated with cryotherapy located on her left nasal alae for an initial diagnosis of actinic keratosis and diagnosed as TLC at last surgery was referred for RT of microscopic surgical margins. The patient was treated with 6 MeV electron beam RT prescribed to surgical bed plus 1-cm margins at all directions, namely the planning target volume. The total and per fraction doses were 60 and 2 Gy, respectively, which is commonly practiced for any skin tumor with positive margins. The treatment was well tolerated with no acute or chronic complications. The patient was alive with no local, regional, or distant recurrences at the 49 months of her follow-up. Conclusions: Although the follow-up period is relatively short and further evidence is needed to confirm the exact role of RT in adjuvant treatment of TLC, the outcomes of present rare case of a nasal TLC suggests that adjuvant RT in patients with positive surgical margins may provide satisfactory local tumor control.
{"title":"Adjuvant radiotherapy in management of trichilemmal carcinoma of left nasal alae with positive surgical margins: A case report","authors":"E. Topkan, O. Guler, N. Bal, Y. Ozdemir","doi":"10.5430/CRCP.V3N4P57","DOIUrl":"https://doi.org/10.5430/CRCP.V3N4P57","url":null,"abstract":"Background: Trichilemmal carcinoma (TLC) is a rare adnexal malignant tumor developing from the outer root sheath of hair follicles with no distinct clinical features which might clinically be misdiagnosed as actinic keratosis, nodular melanoma, basal or squamous cell carcinoma. Albeit no consensus exists on standard treatment of TLC, tumor excision with adequate clear margins is the current widely accepted treatment consideration with no previous literature on use of radiotherapy (RT) in definitive or postoperative settings. Case presentation: A 60-year-old woman who was repeatedly treated with cryotherapy located on her left nasal alae for an initial diagnosis of actinic keratosis and diagnosed as TLC at last surgery was referred for RT of microscopic surgical margins. The patient was treated with 6 MeV electron beam RT prescribed to surgical bed plus 1-cm margins at all directions, namely the planning target volume. The total and per fraction doses were 60 and 2 Gy, respectively, which is commonly practiced for any skin tumor with positive margins. The treatment was well tolerated with no acute or chronic complications. The patient was alive with no local, regional, or distant recurrences at the 49 months of her follow-up. Conclusions: Although the follow-up period is relatively short and further evidence is needed to confirm the exact role of RT in adjuvant treatment of TLC, the outcomes of present rare case of a nasal TLC suggests that adjuvant RT in patients with positive surgical margins may provide satisfactory local tumor control.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"3 1","pages":"57"},"PeriodicalIF":0.0,"publicationDate":"2016-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/CRCP.V3N4P57","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210237","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
D. Surprenant, Karlee Novice, Michael B Dreifke, George Garib, J. Swan
Hemosiderotic dermatofibromas (HDFs) are a rare variant of the relatively common dermatofibroma (DF). HDFs can clinically mimic melanoma as well as other melanocytic lesions and are diagnosed with histopathological examination. Histopathologic diagnosis is not straightforward as there are many DF variants and the features of several variants can often be found in one lesion. These lesions may represent a stage in development from common DF to aneurysmal dermatofibroma (ADF). This possible progression is important to recognize, as ADFs tend to exhibit increased tendency for local recurrence and can also be confused with malignant lesions. We present a case of a 65-year-old presenting with a rare HDF having unique clinical and dermatoscopic findings. Our case underscores the importance of recognizing this rare entity and stresses the importance of remaining mindful of the possible progression to ADF, an entity with increased propensity for recurrence.
{"title":"Hemosiderotic dermatofibroma: Unique clinical presentation and dermoscopic findings of a rare dermatofibroma variant","authors":"D. Surprenant, Karlee Novice, Michael B Dreifke, George Garib, J. Swan","doi":"10.5430/CRCP.V3N4P53","DOIUrl":"https://doi.org/10.5430/CRCP.V3N4P53","url":null,"abstract":"Hemosiderotic dermatofibromas (HDFs) are a rare variant of the relatively common dermatofibroma (DF). HDFs can clinically mimic melanoma as well as other melanocytic lesions and are diagnosed with histopathological examination. Histopathologic diagnosis is not straightforward as there are many DF variants and the features of several variants can often be found in one lesion. These lesions may represent a stage in development from common DF to aneurysmal dermatofibroma (ADF). This possible progression is important to recognize, as ADFs tend to exhibit increased tendency for local recurrence and can also be confused with malignant lesions. We present a case of a 65-year-old presenting with a rare HDF having unique clinical and dermatoscopic findings. Our case underscores the importance of recognizing this rare entity and stresses the importance of remaining mindful of the possible progression to ADF, an entity with increased propensity for recurrence.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"3 1","pages":"53"},"PeriodicalIF":0.0,"publicationDate":"2016-08-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/CRCP.V3N4P53","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210208","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Perineuroma is a rare tumor originating from perineurium of the peripheral nerve. In contrast to neurofibroma and schwannoma, both of which arise from endoneurium and are immunohistochemically positive for S100 protein, perineuroma is negative for S100 protein; the fact makes the pathologic diagnosis difficult. As is well known, stem cells are highly associated with neuronal and organ developments in human embryos, and some neuronal cells are suspected to be derived from neuronal stem cells. Herein reported a very rare case of subcutaneous perineuroma negative for S100 protein but positive for a number of stem cell antigens; the case suggested that some perineuromas could arise from neuronal stem cells. A 68-year-old woman presented with a skin tumor of 10 mm in diameter in the foot. Physical examination revealed a small movable subcutaneous tumor, and resection was performed. Histologically, the tumor was a well-defined round tumor measuring 0.9 cm × 0.9 cm × 0.9 cm located in the subcutaneous tissue. No capsule was seen. The tumor was composed of hypocellular myxoid, neuroid tissues containing small areas of high cellularity where tumor cells resembled neuronal stem cells. Immunohistochemically, the tumor was negative for S100 protein, HMB-45, various cytokeratin (CK), EMA, α-SMA, desmin, p53, and many other antigens. The neuronal stem cell-like cells were positive for various stem cell antigens including NSE, KIT, bcl-2, chromogranin, synaptophysin, PDGFRA, MET, ErbB2, and CD34, as well as for vimentin and Ki-67 antigen (labeling index = 1.5%). Because the tumor was negative for S100 protein but positive for neuroendocrine molecules and neuronal stem cell antigens, the author diagnosed the tumor as perineuroma with stem cell features. Such a perineuroma has not been reported to date. The outcome of the patient was excellent.
摘要神经周围瘤是一种罕见的肿瘤，起源于周围神经的神经周围膜。神经纤维瘤和神经鞘瘤都起源于神经内膜，免疫组织化学上S100蛋白呈阳性，与之相反，神经周围瘤的S100蛋白呈阴性;这一事实使病理诊断变得困难。众所周知，干细胞与人类胚胎的神经和器官发育密切相关，一些神经细胞被怀疑来源于神经干细胞。本文报告一例非常罕见的皮下周围神经瘤，S100蛋白阴性，但许多干细胞抗原阳性;该病例提示一些周围瘤可能起源于神经干细胞。一位68岁的女性，在足部出现了直径10毫米的皮肤肿瘤。体格检查发现一个小的可移动的皮下肿瘤，并进行了切除。组织学上，肿瘤位于皮下组织，大小为0.9 cm × 0.9 cm × 0.9 cm，圆形，轮廓清晰。没有看到胶囊。肿瘤由低细胞黏液组成，神经组织含有小区域的高细胞性，肿瘤细胞类似于神经干细胞。免疫组化结果显示，肿瘤S100蛋白、HMB-45、多种细胞角蛋白(CK)、EMA、α-SMA、desmin、p53等多种抗原均阴性。神经干细胞样细胞NSE、KIT、bcl-2、chromogranin、synaptophysin、PDGFRA、MET、ErbB2、CD34等多种干细胞抗原及vimentin、Ki-67抗原阳性(标记指数为1.5%)。由于肿瘤中S100蛋白呈阴性，而神经内分泌分子和神经干细胞抗原呈阳性，笔者诊断为具有干细胞特征的周围神经瘤。这种周围瘤至今未见报道。病人的预后很好。
{"title":"Perineuroma of the skin negative for S100 protein but positive for stem cell antigens: A possible stem cell tumor","authors":"T. Terada","doi":"10.5430/crcp.v4n1p13","DOIUrl":"https://doi.org/10.5430/crcp.v4n1p13","url":null,"abstract":"Perineuroma is a rare tumor originating from perineurium of the peripheral nerve. In contrast to neurofibroma and schwannoma, both of which arise from endoneurium and are immunohistochemically positive for S100 protein, perineuroma is negative for S100 protein; the fact makes the pathologic diagnosis difficult. As is well known, stem cells are highly associated with neuronal and organ developments in human embryos, and some neuronal cells are suspected to be derived from neuronal stem cells. Herein reported a very rare case of subcutaneous perineuroma negative for S100 protein but positive for a number of stem cell antigens; the case suggested that some perineuromas could arise from neuronal stem cells. A 68-year-old woman presented with a skin tumor of 10 mm in diameter in the foot. Physical examination revealed a small movable subcutaneous tumor, and resection was performed. Histologically, the tumor was a well-defined round tumor measuring 0.9 cm × 0.9 cm × 0.9 cm located in the subcutaneous tissue. No capsule was seen. The tumor was composed of hypocellular myxoid, neuroid tissues containing small areas of high cellularity where tumor cells resembled neuronal stem cells. Immunohistochemically, the tumor was negative for S100 protein, HMB-45, various cytokeratin (CK), EMA, α-SMA, desmin, p53, and many other antigens. The neuronal stem cell-like cells were positive for various stem cell antigens including NSE, KIT, bcl-2, chromogranin, synaptophysin, PDGFRA, MET, ErbB2, and CD34, as well as for vimentin and Ki-67 antigen (labeling index = 1.5%). Because the tumor was negative for S100 protein but positive for neuroendocrine molecules and neuronal stem cell antigens, the author diagnosed the tumor as perineuroma with stem cell features. Such a perineuroma has not been reported to date. The outcome of the patient was excellent.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"4 1","pages":"13"},"PeriodicalIF":0.0,"publicationDate":"2016-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/crcp.v4n1p13","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
Primary neuroendocrine carcinoma (NEC) except for Merkel cell carcinoma has rarely been reported in the skin. Herein reported is a unique case of a primary cutaneous NEC with histological features of squamous cell carcinoma (SCC) with high cellular anaplasia; the tumor is called “squamous NEC” in this report. A 77-year-old man presented with a skin tumor measuring 0.7 cm × 0.8 cm × 0.7 cm of the right second finger. A biopsy showed invasive malignant epithelial cells with keratinization. The carcinoma cells were continuous with epidermis, and showed high cellular anaplasia. No apparent lympho-vascular permeations were seen. The pathological diagnosis was poorly differentiated SCC. Because the histological features were unusual, an immunohistochemical study was performed. The carcinoma cells were positive for cytokeratin (CK) AE1/AE3, CK34BE12, CK5/6, neuron-specific enolase (NSE) (positive percentage (PP) = 70%), chromogranin (PP = 95%), synaptophysin (PP = 95%), NCAM (PP = 20%), p63, p40, S100 protein (Langerhans cells), and Ki-67 (labeling index (LI) = 98%). The carcinoma cells were negative for CK7, CK8, CK9, CK14, CK18, CK19, CK20, CK CAM5.2, smooth muscle actin, vimentin, desmin, HMB45, p53, CD45, TTF-1, HCG, and surfactant apo-protein. Because the skin SCC showed neuroendocrine features and the neuroendocrine cells exceeded 50% of the total tumor cell population, a diagnosis of squamous NEC was made. The histological features, NEC features, high anaplasia, and high Ki-67 labeling index (LI = 98%) suggested poor prognosis. Systemic investigations using CT, MRI, PET, and upper and lower gastrointestinal endoscopy revealed no tumors except for the finger tumor. No lympho-adenopathy was seen. The outcome seemed not good. The tumor of the finger was difficult for operation. Wide excision or finger amputation is considered.
除默克尔细胞癌外，原发性神经内分泌癌(NEC)在皮肤中很少报道。本文报告一例独特的原发性皮肤NEC，其组织学特征为鳞状细胞癌(SCC)伴高细胞间变性;本报告称该肿瘤为“鳞状NEC”。77岁男性，右二指皮肤肿瘤大小为0.7 cm × 0.8 cm × 0.7 cm。活检显示浸润性恶性上皮细胞伴角化。癌细胞连续排列，呈表皮状，呈高度细胞间变性。未见明显淋巴血管浸润。病理诊断为低分化SCC。由于组织学特征不寻常，因此进行了免疫组织化学研究。细胞角蛋白(CK)、AE1/AE3、CK34BE12、CK5/6、神经元特异性烯醇化酶(NSE)(阳性率70%)、色粒蛋白(阳性率95%)、突触素(阳性率95%)、NCAM(阳性率20%)、p63、p40、S100蛋白(朗格汉斯细胞)、Ki-67(标记指数98%)均呈阳性。癌细胞CK7、CK8、CK9、CK14、CK18、CK19、CK20、CK CAM5.2、平滑肌肌动蛋白、vimentin、desmin、HMB45、p53、CD45、TTF-1、HCG、表面活性剂载脂蛋白均阴性。由于皮肤SCC表现为神经内分泌特征，且神经内分泌细胞超过肿瘤细胞总数的50%，因此诊断为鳞状NEC。组织学特征、NEC特征、高分化、Ki-67标记指数高(LI = 98%)提示预后不良。经CT、MRI、PET及上下消化道内镜检查，除手指肿瘤外，未见其他肿瘤。未见淋巴腺病变。结果似乎并不好。手指的肿瘤手术比较困难。考虑广泛切除或手指截肢。
{"title":"Primary squamous neuroendocrine carcinoma of the skin: A case report with immunohistochemical study","authors":"T. Terada","doi":"10.5430/CRCP.V4N1P1","DOIUrl":"https://doi.org/10.5430/CRCP.V4N1P1","url":null,"abstract":"Primary neuroendocrine carcinoma (NEC) except for Merkel cell carcinoma has rarely been reported in the skin. Herein reported is a unique case of a primary cutaneous NEC with histological features of squamous cell carcinoma (SCC) with high cellular anaplasia; the tumor is called “squamous NEC” in this report. A 77-year-old man presented with a skin tumor measuring 0.7 cm × 0.8 cm × 0.7 cm of the right second finger. A biopsy showed invasive malignant epithelial cells with keratinization. The carcinoma cells were continuous with epidermis, and showed high cellular anaplasia. No apparent lympho-vascular permeations were seen. The pathological diagnosis was poorly differentiated SCC. Because the histological features were unusual, an immunohistochemical study was performed. The carcinoma cells were positive for cytokeratin (CK) AE1/AE3, CK34BE12, CK5/6, neuron-specific enolase (NSE) (positive percentage (PP) = 70%), chromogranin (PP = 95%), synaptophysin (PP = 95%), NCAM (PP = 20%), p63, p40, S100 protein (Langerhans cells), and Ki-67 (labeling index (LI) = 98%). The carcinoma cells were negative for CK7, CK8, CK9, CK14, CK18, CK19, CK20, CK CAM5.2, smooth muscle actin, vimentin, desmin, HMB45, p53, CD45, TTF-1, HCG, and surfactant apo-protein. Because the skin SCC showed neuroendocrine features and the neuroendocrine cells exceeded 50% of the total tumor cell population, a diagnosis of squamous NEC was made. The histological features, NEC features, high anaplasia, and high Ki-67 labeling index (LI = 98%) suggested poor prognosis. Systemic investigations using CT, MRI, PET, and upper and lower gastrointestinal endoscopy revealed no tumors except for the finger tumor. No lympho-adenopathy was seen. The outcome seemed not good. The tumor of the finger was difficult for operation. Wide excision or finger amputation is considered.","PeriodicalId":90463,"journal":{"name":"Case reports in clinical pathology","volume":"4 1","pages":"1"},"PeriodicalIF":0.0,"publicationDate":"2016-08-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://sci-hub-pdf.com/10.5430/CRCP.V4N1P1","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"71210629","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
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