Journal of Pathology and Translational Medicine

Case Studies

Mucinous Carcinoma with Extensive Signet Ring Cell Differentiation: A Case Report: Hye Min Kim, Eun Kyung Kim, Ja Seung Koo; J Pathol Transl Med. 2017;51(2):176-179. Published online December 5, 2016; DOI: https://doi.org/10.4132/jptm.2016.08.17

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Breast cancers that present with mucin include mucinous carcinoma and carcinoma with signet ring cell differentiation. The former shows extracellular mucin and the latter shows abundant intracellular mucin. Here, we report a case of breast cancer showing both extracellular mucin and extensive signet ring cell differentiation due to abundant intracellular mucin. Unlike mucinous carcinoma, this case had the features of high-grade nuclear pleomorphism, high mitotic index, estrogen receptor negativity, progesterone receptor negativity, human epidermal growth factor receptor-2 positivity, and ductal type with positivity for E-cadherin. In a case with signet ring cell differentiation, differential diagnosis with metastatic signet ring cell carcinoma of the stomach and colon is essential. In this case, the presence of accompanied ductal carcinoma in situ component and mammaglobin and gross cystic disease fluid protein-15 positivity were findings that suggested the breast as the origin.

Citations

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Research on the Histological Features and Pathological Types of Gastric Adenocarcinoma With Mucinous Differentiation
Nian-Long Meng, Yang-kun Wang, Hai-Li Wang, Jun-Ling Zhou, Su-nan Wang
Frontiers in Medicine.2022;[Epub] CrossRef
Clinicopathologic characteristics of HER2-positive pure mucinous carcinoma of the breast
Yunjeong Jang, Hera Jung, Han-Na Kim, Youjeong Seo, Emad Alsharif, Seok Jin Nam, Seok Won Kim, Jeong Eon Lee, Yeon Hee Park, Eun Yoon Cho, Soo Youn Cho
Journal of Pathology and Translational Medicine.2020; 54(1): 95. CrossRef
Human Epidermal Growth Factor Receptor 2-positive Mucinous Carcinoma with Signet Ring Cell Differentiation, Which Showed Complete Response after Neoadjuvant Chemotherapy
Yunjeong Jang, Eun Yoon Cho, Soo Youn Cho
Journal of Breast Cancer.2019; 22(2): 336. CrossRef

Rare Case of Anal Canal Signet Ring Cell Carcinoma Associated with Perianal and Vulvar Pagetoid Spread: Na Rae Kim, Hyun Yee Cho, Jeong-Heum Baek, Juhyeon Jeong, Seung Yeon Ha, Jae Yeon Seok, Sung Won Park, Sun Jin Sym, Kyu Chan Lee, Dong Hae Chung; J Pathol Transl Med. 2016;50(3):231-237. Published online October 8, 2015; DOI: https://doi.org/10.4132/jptm.2015.08.08

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Abstract PDF

A 61-year-old woman was referred to surgery for incidentally found colonic polyps during a health examination. Physical examination revealed widespread eczematous skin lesion without pruritus in the perianal and vulvar area. Abdominopelvic computed tomography showed an approximately 4-cm-sized, soft tissue lesion in the right perianal area. Inguinal lymph node dissection and Mils’ operation extended to perianal and perivulvar skin was performed. Histologically, the anal canal lesion was composed of mucin-containing signet ring cells, which were similar to those found in Pagetoid skin lesions. It was diagnosed as an anal canal signet ring cell carcinoma (SRCC) with perianal and vulvar Pagetoid spread and bilateral inguinal lymph node metastasis. Anal canal SRCC is rare, and the current case is the third reported case in the English literature. Seven additional cases were retrieved from the world literature. Here, we describe this rare case of anal canal SRCC with perianal Pagetoid spread and provide a literature review.

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A Case of Prostatic Signet-Ring Cell-like Carcinoma with Pagetoid Spread and Intraductal Carcinoma and Long-Term Survival: PD-L1 and Mismatch Repair System Proteins (MMR) Immunohistochemical Evaluation with Systematic Literature Review
Nektarios Koufopoulos, Argyro-Ioanna Ieronimaki, Andriani Zacharatou, Alina Roxana Gouloumis, Danai Leventakou, Ioannis Boutas, Dionysios T. Dimas, Adamantia Kontogeorgi, Kyparissia Sitara, Lubna Khaldi, Magda Zanelli, Andrea Palicelli
Journal of Personalized Medicine.2023; 13(6): 1016. CrossRef
Anal canal adenocarcinoma with neuroendocrine features accompanying secondary extramammary Paget disease, successfully treated with modified FOLFOX6: a case report
Masamichi Yamaura, Takeshi Yamada, Rei Watanabe, Hitomi Kawai, Suguru Hirose, Hiroki Tajima, Masashi Sato, Yuichi Uchida, Daisuke Suganuma, Yoshiyuki Yamamoto, Toshikazu Moriwaki, Ichinosuke Hyodo
BMC Cancer.2018;[Epub] CrossRef
Solitary left axillary lymph node metastasis after curative resection of carcinoma at the colostomy site: a case report
Ken Imaizumi, Shigenori Homma, Tadashi Yoshida, Tatsushi Shimokuni, Hideyasu Sakihama, Norihiko Takahashi, Hideki Kawamura, Emi Takakuwa, Akinobu Taketomi
Surgical Case Reports.2016;[Epub] CrossRef

Case Report

Apocrine Carcinoma of the Axilla with Predominant Signet Ring Cell Features A Case Report.: Jeana Kim, Tae Eun Kim, Ah Won Lee, Yeong Jin Choi, Kyo Young Lee, Eun Sun Jung; Korean J Pathol. 2011;45(3):326-328.; DOI: https://doi.org/10.4132/KoreanJPathol.2011.45.3.326

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Abstract PDF: Apocrine carcinoma arising from the apocrine sweat glands is a rare cutaneous malignant tumor which occurs predominantly in the axilla of elderly individuals. The typical histologic features of apocrine carcinoma is within a well developed glandular lumina with abundant eosinophilic cytoplasm and evidence of decapitation secretion. In rare instances, predominant signet ring cell features in apocrine carcinoma has been reported. We experienced a case that occured in the right axilla of a 59-year-old. Histopathologic examination showed a solid tumor that extended from the upper dermis into the subcutis, with a delicate infiltrate of epithelial cells. The cells had granular amphophilic cytoplasm, predominantly showed distinct signet ring cell morphology, and were strongly positive for epithelial mucin. Both lysozyme and gross cystic disease fluid protein-15 were identified in the tumor cells. We diagnosed this to be a case of primary signet ring cell apocrine carcinoma of the axilla after several immunohistochemical and clinical evaluations.

Original Article

Signet Ring Cell Variant of Invasive Lobular Carcinoma of Male Breast.: Seung Sam Paik, Seok Hoon Jeon, Moon Hyang Park, Pa Jong Jung, Jung Dal Lee; Korean J Pathol. 1997;31(2):179-181.

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Abstract PDF: Lobular carcinoma of the male breast is very rare, because of the absence of lobules in the normal male breast. Herein, a case of lobular carcinoma of the male breast with cellular features of signet ring cells is described. A 57-year-old man presented with a left breast mass. Histologic examination showed classic invasive lobular carcinoma with in situ component. Most infiltrating tumor cells had a prominent signet ring cell appearance. The patient was phenotypically male and had fathered children. There was no history of predisposing factors to breast lesion, such as hormone use or gynecomastia.

Case Reports

Signet Ring Cell Carcinoma of the Prostate A report of two cases.: Yu Na Kang, Sang Sook Lee, Tae Jin Lee, Jae Yoon Ro; Korean J Pathol. 1999;33(5):385-368.

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Abstract PDF: Primary signet ring cell carcinoma of the prostate is extremely rare and about 18 cases have been reported in the literature. We report two cases of primary signet ring cell carcinoma of the prostate, arising in 79-year-old and 65-year-old men. Both cases were the poorly differentiated adenocarcinoma of the prostate with many signet ring cells. Signet ring cells were positive for prostatic specific antigen and prostatic acid phosphatase but negative for neutral and acid mucins. In summary, the signet ring cell carcinoma of the prostate is a rare variant of poorly differentiated adenocarcinoma of the prostate. The orgin of the prostate should be considered in cases of metastatic signet ring cell carcinoma, particularly when the signet ring cells are negative for neutral and acid mucins. Prostatic specific antigen and prostatic acid phosphatase should also be performed to confirm the primary signet ring cell carcinoma of the prostate.

Primary Signet Ring Cell Carcinoma of the Lung: Report of Two Cases .: Dong Ja Kim, Sook Hee Lee, Yoon Kyung Sohn; Korean J Cytopathol. 1997;8(1):83-86.

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Abstract PDF: Signet ring cell carcinoma is a variant of adenocarcinoma and has been rarely reported in the lung as a primary site. Recently, we experienced two cases of primary signet ring cell carcinoma in the lung without any other extrapulmonary lesion. Sputum cytology was performed and the tumor cells which have eccentrically located nuclei and abundnat mucinous cytoplasm were dispersed in diffuse sheets. On resected specimen, the signet ring cells occupied about 50~80% of all tumor cell nests. HIstochemical staining revealed that the mucin produced by tumor cells was mostly carboxylated acid mucins. Ultrastructurally, the tumor cells contained variable sized membrane-bound mucin granules with weak central osmiophilic density and showed numerous surface microvilli, which represented that tumor cells arose from bronchial epithelial cells. In general, this tumor has diffusely infiltrative nature and the prognosis is fatal due to widespread metastasis before clinical discovery.

Urinary Cytologic Findings of Small Cell Neuroendocrine Carcinoma: A Case Report.: Dong Hoon Kim, Dong Wook Kang, Kyung Hee Kim, Ju Heon Kim, Mee Ja Park; Korean J Cytopathol. 2002;13(2):78-83.

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Abstract PDF: We report the cytologic features of a case of primary small cell carcinoma of the urinary bladder with high grade transitional cell and signet ring cell carcinomatous components. A 64-year-old male presented with gross hematuria for one week. Computed tomography revealed an ill-defined mass in the left lateral wall of the urinary bladder. Urinary cytology showed hypercellularity with predominantly isolated single cells and clustered cells. They have scanty cytoplasm and naked hyperchromatic nuclei with finely granular nuclear chromatin and rare nucleoli. The tumor cells occurred predominantly singe cells, but a few in clusters. Nuclear molding was prominent. No glandular formation or nesting was noted. The second tumor cells had high nuclear/cytoplasmic ratio, irregular nuclear membrane, and coarse granular chromatin. The background was inflamed and necrotic. The histologic findings of transurethral resection were mainly composed of small cell carcinoma, and partly transitional cell and signet ring cell carcinomatous components. Small cell neuroendocrine carcinoma have distinctive cytologic features to make a proper diagnosis.

Two Cases of Invasive Carcinoma of the Breast Composed Mostly of Signet Ring Cells in the Fine Needle Aspiration Cytology.: Won Mi Lee, Wan Sup Kim, Eun Kyung Kim, Jong Eun Joo; Korean J Cytopathol. 2002;13(2):88-92.

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Abstract PDF: Scattered single cells or variable sized clusters of signet ring cells in the aspirated smears of breast lesions are almost exclusively associated with carcinoma. The signet ring cells are defined as those containing a prominent intracytoplasmic vacuole or amorphous cytoplasm diffusely dispersed with mucin. The primary signet ring cell carcinoma of the breast behaves more aggressively than carcinoma without signet ring cells. Therefore, it is very important to make a correct diagnosis of signet ring cell carcinoma. Fine needle aspiration cytology is useful for diagnosis of breast lesions including signet ring cell carcinoma. We report two cases, which showed mostly signet ring cells in the aspirated smears of the breast. One case consisted of numerous individual signet ring cells and variable sized cell clusters in rather mucoid background. The tumor cells had abundant amorphous cytoplasm filled with dispersed mucin or occasionally mucin vacuoles(PAS +) and eccentric nuclei. The resected mass revealed mucinous carcinoma. The other showed the cytologic findings of low cellularity, and small loosely cohesive signet ring cell clusters with mild nuclear pleomorphism. It was confirmed as lobular signet ring cell carcinoma in the resected tumor.

Cytologic Features of Signet Ring Cell Carcinoma of the Uterine Cervix: A Report of Two Cases.: Hyun Yee Cho, Seung Yeon Ha, Jaegul Chung, Young Ha Oh, Dong Hae Chung, Na Rae Kim, Jong Min Lee, Eui Don Lee; Korean J Cytopathol. 2003;14(2):66-70.

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Abstract PDF: Signet ring cell carcinoma is a rare type of mucinous adenocarcinoma of the uterine cervix. To the best of our knowledge, there is no report on cytologic findings of primary signet ring cell carcinoma of the uterine cervix in the literature. Recently, we experienced two cases of signet ring cell carcinoma of the uterine cervix. The finding of characteristic signet ring cells on cervicovaginal smear led to the diagnosis of signet ring cell carcinoma. However, primary signet ring cell carcinoma could not be cytologically distinguished from more common metastatic tumor. Therefore, diagnosis rests upon the recognition of signet ring cells and the absence of signet ring cell carcinoma elsewhere.

Giant Cystic Adenomatoid Tumor of the Uterus: A Case Report.: Mi Jin Kim, Mi Jin Gu; Korean J Pathol. 2004;38(6):415-418.

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Abstract PDF: Adenomatoid tumor of the uterus is a rare benign tumor of the uterine serosa and myometrium. It usually appears as a small nodular lesion having a distinctive histologic appearance. We describe a case of 67-year old female with a large, grossly cystic adenomatoid tumor 7x7 cm in dimension that was located at the right uterine cornus. Microscopically the tumor had unusually extensive solid proliferation of signet ring cells with large vacuoles in their cytoplasm. Also, adenoid and honeycomb-like cystic patterns were observed. The immunohistochemical and ultrastructural studies demonstrated the mesothelial origin.

Signet Ring Cell Carcinoma of Stomach in Child: Report of a case.: Kyeong Mee Park, Ill Hyang Ko; Korean J Pathol. 1994;28(1):102-105.

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Abstract PDF: Cancer of the alimentary tract in children is an extremely rare occurence, and it accounts for no more than 5% of all pediatric neoplasms. Malignant neoplasms of the stomach are particularly uncommon in childhood. The majority of such lesions are malignant lymphomas or soft tissue sarcomas, with less than 5% identified as carcinomas. We report a case of signet ring cell carcinoma of stomach in 15-year-old girl. This patient had a clinical manifestation of cough with sputum, fever, abdominal distension due to ascites, hepatomegaly, and cervical lymphadenopathy for 40 days. There was no known gastrointestinal symptoms. Metastatic signet ring cell carcinoma was suspected by aspiration biopsy cytology of cervical lymph node, and confirmed later by excisional biopsy. Endoscopy confirmed a small mucosal erosion in the anterior wall of the antrum and histopathology confirmed the diagnosis of signet ring cell carcinoma.

Original Articles

Cytologic Heterogeneity of Signet Ring Cell Carcinoma of the Stomach: Histochemical and electron microscopic observations.: Yun Kyung Kang, Yong Il Kim; Korean J Pathol. 1992;26(5):427-435.

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Abstract PDF: The cytologic heterogeneity of the tumor cells in gastric signet ring cell carcinoma was studied with 13 surgically early gastric carcinoma specimens by means of histochemical stainings on mucin(periodic acid Schiff-alcian blue at pH 2.5, paradoxical concanavalin A, high iron diamine) and electron microscopy. Of the 13 cases of early gastric cell carcinomas, 6 were mucosal type and 7 were submucosal type. Eleven cases consisted of mixture of gastric and intestinal type signet ring cells and the remaining 2 of the mucosal type were entirely made of gastric type. The colonic goblet cell type was found in 4 of the submucosal type. Within the mucosa the tumor cells showed a layering phenomenon; type A signet ring cells were distributed at the central zone and type B and C at the superficial or deeper zone. Each type of signet ring cell showed variable mucin histochemical stainability of gastric and/or intestinal nature. Above finding strongly suggest that the variable phenotypes of signet ring cells result from a heterogeneity of cytoplasmic mucin as well as different stages of differentiation of signet ring carcinoma cells.

Primary Signet Ring Cell Carcinoma with Widespreand Metastasis.: Nam Hoon Cho, Soon Hee Jung, Tae Sung Kim; Korean J Pathol. 1988;22(4):448-455.

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Abstract PDF: We present an autopsy case of signet ring cell carcinoma of urinary bladder which underwent widespread metastsis through nearly entire organ. The patient, 45-year old male, suffered from hematuria, dysuria and flank pain for 6 months. Under the diagnosis of the signet ring cell carcinoma by the cystoscopic biopsy, radical cystectomy and ileocutaneous ureteral division were performed. But he died after 11-months after the diagnosis. The autopsy confirmed the widespread metastasis at the lung, kidney, adrenal gland, parathyroid gland, heart, stomach, ileum, spine, rib and meninges. The preveously resected urinary bladder discolosed the well defined fungating mass at the dome and anterio-lateral portion without apparent evidence of the remnant of the urachus. Histologically, the signet ring cells were predominent and focally mucinous pool floating the signet ring cells was noted. We could find several foci of the well differentiated adenocarcinoma and poorly differentiated adenocarcinomas. The above findings may reflect that the origin of carcinoma may be from the multipotential transitional cell epithelium.

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