scholarly journals Microcystic adnexal carcinoma: report of rare cases

2020 ◽  
Vol 40 (1) ◽  
Author(s):  
Lei Zhang ◽  
Xiaofeng Huang ◽  
Ting Zhou ◽  
Hongbao Cao

Abstract Microcystic adnexal carcinoma (MAC) is a rare, locally aggressive malignant neoplasm that derives from cutaneous eccrine/apocrine glands. MAC is classified as an eccrine/apocrine gland tumor and usually occurs in the skin. Here, we characterized and compared two cases of MAC. One is extremely rare in terms of its occurrence in the tongue. The other occurred in the lip, which is common. Histories of disease, diagnosis, and differentials were reviewed by the attending physicians. Hematoxylin and Eosin (HE) slides were evaluated by an experienced pathologist. Immunological markers for malignant eccrine/apocrine gland tumors were used to characterize the tumor’s nature. The examined markers included EMA, CK5/6, CK8/18, CK7, CK20, p63, S-100, Calponin, CD10, MYB, Bcl-2, Her-2, CD34, SMA, p53, CD43, CD117, and Ki-67. Both patients were males, presented with painless lumps in the lower lip and in the tongue, respectively. Both lumps were similar in terms of appearance, being whitish, and infiltrative with irregular borders. Both tumors also had similar histological features with nests of bland keratinocytes, cords, and ductal differentiation filled with Periodic acid–Schiff (PAS)-positive eosinophilic material. In both cases, circular or ovary tumor cells invaded into muscles and nerves. All tumor cells were CK5/6, CK8/18, EMA, and CK7 positive. Particularly, keratinocytes were p63 positive, and paraductal cells were p63, S-100, and SMA positive. Therefore, the rare case of MAC in the tongue appears to derive from the salivary gland.

2018 ◽  
Vol 12 ◽  
pp. 117955491881353
Author(s):  
Yanling Zhang ◽  
Heng Tang ◽  
Huaiyuan Hu ◽  
Xiang Yong

Primary leiomyomas of the thyroid are very rare. We here report a case of a 53-year-old woman with a painless mass at the right thyroid, revealed by physical examination. The patient underwent a lobectomy. Frozen sections showed a spindle cell tumor of the thyroid gland. The nuclei of some of the tumor cells were obviously enlarged and deeply stained. Pseudocapsule invasion was observed in small foci. Samples showed neither mitosis nor necrosis and the nature of the tumor was difficult to determine. Paraffin sections showed a well-circumscribed nodular composed of intersecting fascicles of spindled to slightly epithelioid cells with eosinophilic cytoplasm and blunt-ended, cigar-shaped nuclei. We observed no significant nuclear atypia, mitotsis, or necrosis. Immunohistochemical staining showed the tumor cells to be positive for α-smooth muscle actin and h-caldesmon but negative for TG, TTF1, PAX8, S-100, CT, CK, and CD34. The ki-67 index was very low (<1%). Primary thyroid leiomyoma is rare and difficult to diagnose using frozen sections. Diagnosis requires immunohistochemical staining. Leiomyoma may be mistaken for other thyroid tumors also characterized by spindle cells.


2021 ◽  
Vol 14 (1) ◽  
Author(s):  
Hong Ye ◽  
Min Lin ◽  
Ruotong Li ◽  
Shuming Qin ◽  
Gang Hou ◽  
...  

AbstractThe diagnosis of primary angiosarcoma of ovary is still a challenge as it has no specific clinical symptoms and is easily confused with other malignant neoplasms in morphology. Here, we described a case of primary ovarian angiosarcoma and reviewed the literature. A 47-year-old female showed a left ovary mass. Grossly, the cut surface of the tumor was solid and gray-white with intermediate texture. Some areas were spongy and atropurpureus with a soft texture. Microscopically, the tumor cells were arranged into a variety of different structures with visible hemorrhage. Immunochemically, the tumor cells were positive for CD31, ERG, Fli1, D2–40 and vimentin in a strong and diffused manner. CD34 stain showed focal positivity. Epithelial markers (e.g. CK, CK7, CK8/18 and PAX8) were all negative. Negative immunostaining for SMA, S-100, P53 and calretinin also were detected. The proliferative index (Ki-67) was approximately 40%. After surgery, the patient was treated with radiotherapy, targeted therapy and immunotherapy. In the 9-month follow-up, the patient was survival without evidence of disease. The diagnosis of ovarian angiosarcoma required the careful observation of morphology and the reasonable application of immunohistochemistry. Targeted therapy and immunotherapy are the potential directions for the treatment of angiosarcoma.


2020 ◽  
Author(s):  
Xiaoxia Gou ◽  
Yanzhe Wang ◽  
Fang Chen ◽  
Xiaoli Liu ◽  
Peigang Ruan ◽  
...  

Abstract BackgroundPrimary sinonasal renal cell-like adenocarcinoma (SNRCLA) is an extremely rare neoplasm with unique clinical and pathological features. At present, there is no summary of its clinical and pathological characteristics. We treated one case and reported to here. Review the domestic and foreign literature, summarize its clinical pathological characteristics and diagnosis and treatment.CaseA 69-year-old female presented with repeated epistaxis, nasal obstruction of 2-years’ duration. Computed tomography (CT) was performed revealing an irregular mass of the right nasal cavity involving choana and nasopharynx. The patient was treated with endonasal endoscopic resection. Histologically, SNRCLA was very similar to clear cell renal cell carcinoma (RCC), the tumor cells were round or polygonal in size with abundant clear cytoplasm and uniform nuclei. The tumor cells positive for CK7, Vimentin, EMA, S-100, Ki-67 (5% +) and negative for CEA, P53, P63 by immunohistochemical staining,. The patient is free from recurrence over 27 months after the surgery.ConclusionSNRCLA seems to be a low-grade localized carcinoma associated with favorable prognosis. Surgical resection is recommended as the main stay of treatment.


2021 ◽  
Author(s):  
Mariateresa Ambrosino ◽  
pasquale somma ◽  
andrea santarelli ◽  
stefania staiabano ◽  
michele di cosola ◽  
...  

Abstract Background Sebaceous carcinoma (SC) is a very rare, aggressive, malignant tumor arising in the adnexal epithelium of the sebaceous gland. SC in the oral cavity is extremely rare, in literature there are only 14 cases. We reported the 4th case of sebaceous carcinoma involving the lip Case presentation A 71-year-old male smoker presented an ulcerated lesion in the lateral region of the lower lip. The patient stated that the lesion had been present for 1 year. The past medical history was unremarkable. Extra-oral examination revealed a markedly ulcerated, exophytic, irregularly shaped, indurated mass of the lower right labial region, measuring 1.8 cm in size. The nodular lesion, located at the point of transition between mucosa and skin, showed a central ulceration. No other intraoral lesions were identified. The clinical differential diagnosis included squamous cell carcinoma, basal cell carcinoma with sebaceous differentiation and salivary gland neoplasms. The operation was performed under local anaesthesia. On histopathological examination, the tumor was composed by nodules or sheet of cells separated by a fibrovascolar stroma. The neoplastic tissue was deeply infiltrating, involving the submucosa and even the underlying muscle. Neoplastic cells showed a range of sebaceous differentiation with finely vacuolated rather than clear cytoplasm. Neoplastic cells were positive for S-100 protein, EMA, but negative for CEA. Therefore, based on these findings, a diagnosis of sebaceous carcinoma of the lower lip was rendered. Conclusion The histogenesis, differential diagnosis, and clinicopathologic conditions of this disease in the literature were reviewed. SC should be distinguished from other tumors full of vacuolated clear cells. Some useful biomarkers can be Ki-67, P53, CK, PAS, S-100, EMA, and AR.


2021 ◽  
Vol 7 (1) ◽  
Author(s):  
Yuri Terunuma ◽  
Kazuhiro Takahashi ◽  
Manami Doi ◽  
Osamu Shimomura ◽  
Yoshihiro Miyazaki ◽  
...  

Abstract Background Primary liposarcoma arising from the liver is exceedingly rare. There have been very few reports documenting primary hepatic liposarcoma, especially of the pleomorphic subtype. Surgery is currently the only established treatment method, and the prognosis remains poor. In this report, we present an unusual case of hepatic liposarcoma of the pleomorphic subtype with literature review. In addition, we discuss theories regarding pathogenesis and the pathological and clinical features of primary hepatic liposarcoma to better outline this rare entity. Case presentation An asymptomatic 65-year-old female was found to have a right hepatic mass on a computed tomography scan 2 years after surgical resection of the left adrenal gland and kidney for adrenocortical carcinoma. Laboratory examinations were unremarkable. Magnetic resonance imaging demonstrated a 16-mm mass in the right hepatic lobe. Adrenocortical carcinoma metastasis was suspected. Laparoscopic partial hepatectomy completely removed the tumor with clear margins. Macroscopically, the surgical specimen contained a nodular, yellow–white mass lesion 20 mm in diameter. On pathologic examination, pleomorphic, spindle-shaped tumor cells containing hypochromatic, irregularly shaped nuclei of various sizes formed fascicular structures. Scattered lipoblasts intervened in varying stages. Mitotic cells were frequent. Ki-67 labeling index was 15%. Immunohistochemically, the tumor cells were diffusely positive for vimentin and focally positive for CD34 and alpha-SMA; lipoblasts were focally positive for S-100. Tumor cells were nonreactive for SF-1, inhibin alpha, desmin, HHF35, HMB45, Melan A, MITF, c-kit, DOG1, cytokeratin AE1/AE3, h-caldesmon, STAT6, CD68, MDM2, CDK4, c17, DHEAST, 3BHSD, CD31, Factor 8, and ERG. From these findings, primary hepatic liposarcoma of pleomorphic subtype was diagnosed. The tumor recurred intrahepatically 3 years later, and the patient died 5 months after recurrence. Conclusions In our report, we discussed the rarity, theories regarding pathogenesis, and a review of the literature of this atypical condition. To the best of our search, this is the 14th case of primary hepatic liposarcoma and the 2nd case of the pleomorphic subtype reported throughout the world. Further research regarding the etiology of this unusual clinical entity is warranted to establish effective diagnostic and management protocols.


2021 ◽  
Author(s):  
Jianjun Wang ◽  
Zhong Zheng ◽  
Feng-nan Niu ◽  
Ting Wang ◽  
Biao Zhang ◽  
...  

Abstract Background Systemic mastocytosis is a rare disease and most patients have pigmented urticarial skin lesions. It can be easily missed and misdiagnosed in small biopsies, especially in those patients with nonspecific clinical complaints or untypical skin lesions. Case presentation We report a case of 69-year-old man who have presented with 2-year of diarrhea, progressive weight loss of 20kg and abdominal distention for 3 months. Ultrasound and abdominal CT scan showed massive effusions in abdominal and pelvic cavity. Colonoscopy was performed and showed intensive mucosal proliferations forming polypoid appearances. Microscopically, monotonously uniform, small, round tumor cells with slightly rich cytoplasm were concentrated between the residual glands in the colonoscopic biopsy. The tumor cells showed positive expression of CD117 and S-100, and low Ki-67 proliferation index of 2%, while Trypsin, CK, CD68, CD1a, Langerin, Syn, CgA, CD56, SATB2, CD20, CD3, α-inhibin and SMA were all negative. KIT D816V mutation was detected as well. Liver biopsy showed that CD117 positive cells were more than 15 cells in aggregates around the hepatic portal area and less than 15% mast cells were found in bone marrow smears. No multiple or diffuse pattern of mast cells infiltration was seen by repeated skin biopsies of skin lesions. With all these considered, the diagnosis of aggressive systemic mastocytosis was made. Conclusions The diagnosis of aggressive systemic mastocytosis is challenging for untypical clinical manifestations and subtle or inconspicuous lesions, especially in endoscopic biopsies, which requires awareness and a close teamwork of pathologists and clinicians.


2013 ◽  
Vol 2013 ◽  
pp. 1-5 ◽  
Author(s):  
Kiyoshi Shingu ◽  
Tokiko Ito ◽  
Gengo Kaneko ◽  
Nobuo Itoh

Acinic cell carcinoma of the breast is an extremely rare, malignant neoplasm characterized by widespread acinar cell-like differentiation and clinically low-grade malignancy. Herein, we report a case of acinic cell carcinoma of the breast in a 41-year-old woman. The tumor was poorly demarcated but had a firm consistency. It was removed with lumpectomy, and sentinel lymph node biopsy was performed to check for metastasis. Microscopically, the tumor showed an infiltrative growth pattern with a combination of solid, trabecular, and microglandular areas. Many of the tumor cells had abundant clear vacuolated cytoplasm containing zymogen-typed granules which resemble acinar cells of the salivary glands. The immunohistochemical profile of the tumor was also similar to that of salivary gland acinic cell carcinoma: the tumor cells were positive for amylase, lysozyme,α-1-antichymotrypsin, S-100 protein, and epithelial membrane antigen and negative for estrogen receptor, progesterone receptor, and human epidermal growth factor receptor 2. She received postoperative chemoradiation therapy and has been well for 3 years since surgery. As studies on large series are lacking, further studies are needed to elucidate the biological characteristics of acinic cell carcinoma of the breast.


2017 ◽  
Vol 10 ◽  
pp. 117955571668467
Author(s):  
Yan-jun Dong ◽  
Zhi-wen Zhang ◽  
Zhen Wang ◽  
Xin-ying Wang ◽  
Zhi-zhen Tian ◽  
...  

Background: Pigmented paraganglioma is a special type of paraganglioma, and it is rare in the thyroid. Case Presentation: We report a case of a 41-year-old woman who had complained of a mass in the thyroid gland. Histology revealed tumor cells arranged in a nest-like or organoid pattern, separated by delicate fibrovascular septa. Two distinct components were observed. In the first, which constituted the majority of the tumor cells, no pigments were observed. In the second, a few cells with pigment showed intercellular substance, but the structure was unclear. Using immunohistochemistry, cells in the first component were confirmed to be diffuse strong positive for synaptophysin, but negative for chromogranin A, pan-cytokeratin, calcitonin, and thyroglobulin. About 1% of tumor cells were stained by Ki-67. In the margins of the tumor, a few cells were observed to be positive for HMB-45 and Melan A after bleaching by oxalic acid. The stromal cells were positive for S-100. Using electron microscopy, a few cells containing many round melanin bodies with greater electron density granules of nonuniform size were observed. The diagnosis of primary melanotic paraganglioma of the thyroid gland was made. Conclusion: Primary melanotic paraganglioma of the thyroid gland is a rare, low malignant potential tumor. To the best of our knowledge, this is the first case described.


2021 ◽  
Vol 91 (1) ◽  
pp. 51-63
Author(s):  
Marko Hohšteter ◽  
◽  
Ivan-Conrado Šoštarić-Zuckermann ◽  
Ivana Mihoković Buhin ◽  
Lidija Medven Zagradišnik ◽  
...  

A histopathological analysis of 13 apocrine gland tumors and 22 sebaceous gland tumors in dogs was performed, which were submitted to the Department of Veterinary Pathology from 1 January 2010 to 31 December 2012. An association between the immunohistochemical expression of E-cadherin and Ki-67, the type, and the biological behavior of the tumors was investigated. The mean age of dogs with apocrine gland tumors was 10.15 years, and 10.91 years for sebaceous gland tumors. According to the histopathological analysis, 53.8% carcinomas and 46.2% adenomas of the apocrine glands were found. In the sebaceous gland tumors, epitheliomas were dominant (50.0%), followed by adenomas (36.4%) and carcinomas (13.6%). Survival time in dogs with sebaceous gland tumors was shortest in patients with diagnosed epitheliomas, intermediate in adenomas, and longest in carcinomas. The incidence of metastases was low in the groups with sebaceous adenomas and epitheliomas, and recurrence was most common in carcinomas. The average survival time was about the same for apocrine gland adenomas and carcinomas, with a higher incidence of metastases and recurrence for carcinomas. Immunohistochemical analysis of E-cadherin and Ki-67 expression confirmed the efficiency of this method for the accurate histological classification of apocrine and especially sebaceous gland tumors. The analyzes performed showed that the location and intensity of E-cadherin expression can be helpful in predicting the biological behavior of sebaceous gland tumors. In contrast to sebaceous gland tumors, the analysis of apocrine gland tumors showed that there was no correlation between E-cadherin expression and the biological behavior of apocrine gland tumors.


2021 ◽  
Vol 147 (4) ◽  
pp. 1125-1135
Author(s):  
Sang Kyum Kim ◽  
Jang-Hee Kim ◽  
Jae Ho Han ◽  
Nam Hoon Cho ◽  
Se Joong Kim ◽  
...  

Abstract Purpose Penile carcinoma is a rare malignant neoplasm with a largely unknown molecular pathogenesis. Telomerase reverse transcriptase promoter (TERT-p) mutations have been detected in several types of human malignancies. The aim of this study was to investigate the presence of TERT-p mutations in penile squamous cell carcinomas (SCCs) and their associations with clinicopathologic features. Methods In this retrospective study, Sanger sequencing was performed to detect TERT-p mutations in formalin-fixed paraffin-embedded tissue samples from 37 patients with penile SCC, 16 patients with cutaneous SCC, and 4 patients with non-neoplastic penile/skin tissue. The expression of p16INK4a and Ki-67 was investigated via immunohistochemistry. Associations of TERT-p mutation with clinicopathological factors, immunohistochemical results, and clinical outcome were statistically analyzed. Results Recurrent TERT-p mutations were identified in 18 out of 37 (48.6%) penile SCCs, including all 3 carcinoma in situ cases. TERT-p mutations were significantly more frequent in non-human papilloma virus (HPV)-related penile SCC types than in non-HPV-related penile SCC based on both histologic classification and p16INK4a immunoreactivity. Furthermore, TERT-p mutation was associated with a low histologic grade, low mitotic count, absence of necrosis, low Ki-67/MIB-1 labeling index, and absence of lymph node or distant metastasis. Conclusion Our study shows TERT-p mutations are the most frequent somatic mutations in penile SCC. In addition, TERT-p mutations are far more frequent in non-HPV-related penile SCC than in HPV-related penile SCC, indicating TERT-p mutations may have a role in tumorigenesis distinct from HPV-related penile SCC.


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