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2013 Gastrointestinal Pathology Specialty Conference Handout - Case 3
src: uscapknowledgehub.org

Stem cell ring carcinoma (SRCC) is a rare form of a highly malignant adenocarcinoma that produces mucin. This is an epithelial malignancy characterized by a histologic appearance of a seal ring cell.

Primary SRCC tumors are most commonly found in glandular cells (SRCC originates from the stomach in 90 percent of patients), and less frequently in the breast, gall bladder, bladder, and pancreas. SRCC usually does not form in the lungs, although several incidents have been reported. [1]

Among colorectal cancers, the prevalence of SRCC is less than one percent. Although the incidence and mortality of gastric cancer have declined in many countries over the last 50 years, there has been an increase in the incidence of gastric-type SRCC cancer.

SRCC tumors grow in the characteristic sheets, which make the diagnosis using standard imaging techniques, such as CT scans and PET, are less effective.


Video Signet ring cell carcinoma



Cause

Some cases are inherited, and these cases are often caused by mutations in the CDH1 gene, which encodes important cells of E-cadherin adhesion glycoprotein. Down-regulation E-cadherin is essential for the initiation and development of cancer cells of the stomach seal ring. As soon as these cells lose E-cadherin, their motility increases with epithelial-mesencalal transition. Somatic mutations from the APC gene have also been implicated in the development of gastric SRCC.

The role of other risk factors in stomach cancer such as salt-preserved, smoked, auto-immune gastritis is not well studied in SRCC.

Maps Signet ring cell carcinoma



Formation mechanism

SRCCs are undifferentiated adenocarcinomas that lose the ability to interact with cells.

Highly differentiated adenocarcinomas form the SRCC through loss of adherens and tight joints that normally separate MUC4, mucin protein, and ErbB2, oncogenic receptors. When MUC4 and ErbB2 can interact, they trigger an activation loop. As a result, the ErbB2/ErbB3 signaling pathway becomes actively constitutive, the interaction of the lost cells and the carcinoma of the sign is formed. Constitutive actions of the ErbB2/ErbB3 complex also increase cell growth.

This malignant cancer mechanism remains unclear; However, it has been found that colon carcinoma cells known as HCC2998 cause an increase in the production of differentiated tumors. The reason for this increase is because the active PI3K is converted to an SRCC-like cell.

Signet ring cell cancer in 15 y.o. child. Endosonography uT2N1M1 ...
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Metastasis

Metastatic patterns are different for bell cell carcinoma than for gastric bowel carcinoma. SRCC tumors are often seen in the peritoneum and have also been known to spread to lymphatic lung permeation and to the ovaries, creating a Krukenberg tumor. Cases of gastric carcinoma metastasize to the breast and form mark-cell carriers have also been reported. One study showed that when spiky ring cells are found in breast tumors, the presence of stomach cancer should also be considered.

2013 Gastrointestinal Pathology Specialty Conference Handout - Case 3
src: uscapknowledgehub.org


Histology

The SRCC is named for its resemblance to a pointy ring, which results from the formation of a large vacuole filled with mucin that replaces the nucleus to the periphery of the cell.

Stomach cancer with both adenocarcinoma and some SRC (known as mixed-SRCC) exhibit more aggressive behavior than pure SRCC or non-SRCC histology.

A study of colorectal cancer of SRCC compared SRCC-rich mucosine tumors and poor SRCC tumors. They conclude that the latter more often show adverse histologic features such as lymphatic invasion, venous invasion and perineural invasion.

Signet ring cell - Wikipedia
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Treatment

Chemotherapy has relatively poor curative efficacy in SRCC patients and overall survival rates are lower than in patients with more common cancer disorders. SRCC cancer is usually diagnosed during late stages of the disease, so tumors generally spread more aggressively than non-migrant cancers, making treatment difficult. In the future, case studies show that bone marrow metastasis is likely to play a greater role in the diagnosis and management of gastric stem cell ring cancer.

In the abdominal SRCC, removal of stomach cancer is the treatment of choice. No combination of chemotherapy is clearly superior to others, but the most active regimens include 5-Fluorouracil (5-FU), Cisplatin, and/or Etoposide. Several new agents, including Taxol and Gemcitabine (Gemzar ® ) are under investigation.

In a single case study of patients with SRCC of the bladder with recurrent metastasis, the patient showed a treatment response to the palliative FOLFOX-6 chemotherapy.

Hereditary diffuse gastric cancer: updated clinical guidelines ...
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Prognosis by organ

These aggressive tumors are generally diagnosed at an advanced stage and survival is generally shorter. The SRCC prognosis and its sensitivity to specific regimens are controversial because the SRCC is not specifically identified in most studies and its poor prognosis may be due to a more advanced stage. One study showed that a poor prognosis appears to be due to the biology of its intrinsic tumor, which suggests an area for further research.

Bladder

Carcinoma of the major ring carcinoma cells in the bladder is very rare and patient survival is very bad and occurs mainly in men aged 38 to 83. However, one patient treated with radical cystectomy followed by combined chemotherapy S-1 and adjuvant Cisplatin did demonstrate long-term survival promising length of 90 months.

Colorectal

Carcinoma of colon and rectum main ring stem cells (PSRCCR) is rare, with reported incidents less than 1 percent. It has a poor prognosis because symptoms often develop late and are usually diagnosed at an advanced stage. Five-year survival rates in previous studies ranged from nine to 30 percent. The average survival is between 20 and 45 months. This tends to affect young adults with the possibility of higher lymphovascular invasion. It should be noted that the overall survival rate of patients with SRCC was significantly worse compared with patients with mucinous adenocarcinoma or poorly differentiated.

In advanced gastric cancer, the prognosis for patients with SRCCs is significantly worse than other histologic types, which can be explained by the findings that advanced SRCC stomach cancer has a larger tumor size, more lymph node metastases, deeper and more invasive depth Borrmann 4 type lesions than any other type.

Stomach

When compared with gastric adenocarcinoma, SRCC in the stomach is more common in women and younger patients. Patients with SRCC in the stomach exhibit clinicopathologic features similar to patients with undifferentiated histology. A recent study found that patients with SRCC had a better prognosis than patients with undifferentiated gastric carcinomas. However, when narrowed in patients with advanced stomach cancer alone, those with SRCC have a worse prognosis than other cell types.

Pathology Outlines - Diffuse (poorly cohesive) type adenocarcinoma
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Additional images


Signet Ring Cell Carcinoma of Breast
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References


Pathology Outlines - Diffuse (poorly cohesive) type adenocarcinoma
src: www.pathologyoutlines.com


External links




Source of the article : Wikipedia

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