Primary Colonic Signet Ring Cell Carcinoma Presenting Carcinocythemia: An Autopsy Case

Abstract

Primary colorectal signet ring cell carcinoma (SRCC) is a rare but distinctive type of mucin-producing adenocarcinoma of the large intestine with still controversial clinicopathological features and prognosis. We encountered primary colonic SRCC in a 51-year-old Japanese man with extensive bone metastasis ultimately leading to carcinocythemia before the initiation of chemotherapy and surgical intervention. Three days before death, besides progressive disseminated intravascular coagulation that had been present on admission, hematological examination showed sudden leukocytosis with nonhematopoietic cells that subsequently turned out to be signet ring cells (SRCs). Carcinocythemia, the presence of circulating cancer cells in peripheral blood, is considered to be a rare but an ominous phenomenon occurring in the advanced stage of certain types of cancers, particularly mammary lobular carcinoma. It can be assumed that carcinoma cells lacking intercellular cohesiveness and polarized cell membrane organization, including SRCs as well as lobular carcinoma cells, can readily get access to the peripheral circulation; however, to our knowledge, this is the first report of primary colorectal SRCC that presented carcinocythemia. Extensive bone metastatic sites, in the present case, may have functioned as a reservoir of circulating SRCs.

Carcinocythemia: A rare entity becoming more common? A 3‐year, single institution series of seven cases and literature review

“…They hypothesized that damage to the reticuloendothelial system may prevent these organs from phagocytizing the circulating malignant cells. 6,7 Few authors associated the presence of CTCs with splenectomy or spleen dysfunction, even in enlarged spleens, which they suggest may impair the normal mechanism for clearance of those carcinoma cells. 2,4,[7][8][9] Interestingly, the blood smears of 3 of our patients (cases 1, 4-5) showed asplenic red blood cell morphology with an anatomically intact spleen, similarly suggestive of splenic hypofunction, including one patient with splenomegaly.…”

Treatment of Rectal Cancer-Induced Disseminated Carcinomatosis of the Bone Marrow with FOLFOX plus Cetuximab and Panitumumab

“…In that study, 37 of the 40 cases (92.5%) were due to gastric cancer while one each was due to prostate, gallbladder, and ovarian cancer with unknown primary lesion, but no case of colorectal cancer was reported. A review of the literature revealed that DCBM in colorectal cancer is particularly rare and only 10 cases have been reported previously (Table 1) [8][9][10][11][12][13][14][15][16][17]. The average age of the patients was 61.0 years (men, 9 cases; women, 2 cases).…”

Primary Signet Ring Cell Carcinoma of Rectum Diagnosed by Boring Biopsy in Combination with Endoscopic Mucosal Resection

“…Colorectal SRCC is a very rare subtype, which accounts for only 0.1%–2.6% of all colorectal cancer cases [ 2 ]. In addition to its rarity, colorectal SRCC is considered to have poor prognosis as compared to conventional adenocarcinomas because of its high propensity for diffuse intramural infiltration, lymph node involvement, peritoneal dissemination, and distant metastasis [ 5 ]. In fact, previous age- and sex-matched controlled study has demonstrated that the survival rate of patients with SRCC was significantly lower than that of ordinary adenocarcinomas with independent predictive factors, such as the stage of diagnosis and presence of distant metastasis [ 6 ].…”