30 September 2022: Articles
Rare diseaseZachary J. Naser1ABCDEF*, Shawna Morrissey1ABCDEF
Am J Case Rep 2022; 23:e937548
BACKGROUND: Sarcomatoid carcinoma is a rare tumor that can occur in different organs and anatomical locations. Colonic sarcomatoid carcinoma, also known as carcinosarcoma, is an extremely rare tumor, with only 32 cases reported world-wide. The pathogenesis and guidelines for treatment are poorly understood due to the rarity and invasiveness of the disease.
CASE REPORT: A 77-year-old woman presented with worsening lower abdominal pain and associated fever after having initially been diagnosed with stump appendicitis and associated phlegmon 3 weeks prior, which was treated with antibiotics. Repeat imaging revealed an extraluminal versus perforated colonic mass with associated phlegmon. The patient’s condition continued to worsen, with development of obstructive-like symptoms, resulting in operative intervention involving a R2 right hemicolectomy, stapled ileo-colostomy, and partial omentectomy. The patient had an uneventful remainder of her hospitalization other than continued lower abdominal pain. After initial discharge, the patient presented to an outside hospital due to continued deterioration of health, with findings of an additional mass, likely secondary to the previous lymphadenopathy. Ultimately, goals of care were discussed, and the decision was made to provide palliative care, and the patient died due to her illness 32 days after the initial procedure.
CONCLUSIONS: Carcinosarcoma is an extremely rare tumor with scant research guiding treatment guidelines. Current guidelines gathered from previous case reports suggest treating colorectal carcinosarcoma as adenocarcinoma. Additional research and studies are needed to establish appropriate therapeutic guidelines for carcinosarcoma.
Keywords: Carcinosarcoma, Colorectal Neoplasms, Colorectal Surgery, general surgery, Abdominal Pain, Aged, Anti-Bacterial Agents, Carcinoma, Cellulitis, Colon, Ascending, Female, Humans
Sarcomatoid carcinoma, also referred to as carcinosarcoma, is a rare tumor that exhibits 2 distinct tissue origins. The biphasic tumor has properties composed of epithelial and mesenchymal origin. The epithelial component (carcinoma) is composed of high-grade or mid-grade adenocarcinoma . The mesenchymal component (sarcoma) may or may not be differentiated . These tumors can occur in different anatomic locations or different origins, with the first reported cased in 1864 by Virchow . Over the course of time, research and clinical evidence has shown a predilection for the female genital tract [3,4] along with the head and neck . Isolated reports of intra-abdominal carcinosarcoma have seen higher reports in the stomach  and esophagus  compared to the rest of the gastrointestinal tract. Based upon our review, colorectal carcinosarcoma is extremely rare, with only 32 cases reported in the literature. Herein, we present a case of ascending colon carcinosarcoma treated with a R2 en-block right hemicolectomy, which is resection of all possible gross disease with known residual gross disease that was not resected.
During the procedure, the mass was approximately 7 cm in diameter, involving the ascending colon to the hepatic flexure, with a gross distal margin of 7 cm and a proximal margin of 10 cm. There was no identified involvement of the cecum. The mass was densely adhered to the abdominal sidewall, mesentery, omentum, and duodenum. An area of contained perforation was noted into the mesentery, without feculent or purulent peritonitis. Significant lymphadenopathy was identified in the mesentery down to the root along the superior mesentery artery.
On pathology examination, the specimen was noted to be a biphasic tumor with identified areas of single and multi-nucleated cells with pleomorphic nucleic admixed with spindle cells containing anaplastic nuclei (Figure 5) in addition to areas of pleomorphic nuclei, prominent nucleoli, and abundant mitotic figures (Figure 6). Additional immunohistochemistry performed showed reactive tumor cells to calretinin (Figure 7) but other markers for mesothelioma were negative. Additional tumor cells were positive for claudin-4. The specimen was noted to have extensive area of necrosis measuring 5.7 cm, with extension of the tumor to the peri-colonic fat. Nine lymph nodes were positive malignancy out of 19 (9/19).
The final diagnosis was stage IIIc T4bN2bM0 sarcomatoid carcinoma of the ascending colon involving extension into the mesenteric root and retroperitoneum with negative margins and 9/19 lymph nodes positive for malignancy.
Carcinosarcoma of the colon is an extremely rare tumor. The first reported case, by Weidner and Zekan , was in 1986; it was located in the sigmoid colon and treated with a left hemicolectomy. Since 1986, a total of 32 cases have been reported (Table 1) [1,8–38]. A review of the patient characteristics reported in the literature (Table 2) revealed a mean age of presentation of 67 years old, with 59% of cases in females. The most common chief concern on presentation remains bright red blood per rectum, while abdominal pain is the second most common. The rectum is the site of highest occurrence in the colon, with our case as the sixth reported episode in the ascending colon.
Sarcomatoid carcinoma has had numerous other aliases throughout literature. In addition to sarcomatoid carcinoma, it has been referred to as carcinoma with mesenchymal stroma, carcinoma with sarcomatous change, spindle cell carcinoma, and pleomorphic anaplastic carcinoma . The term carcinosarcoma was officially coined by the World Health Organization as the classification class .
Current knowledge Is lacking in understanding the development of the tumor. Current theory favors a metaplastic origin. It is believed that the carcinoma component differentiates into the sarcomatoid component during the development of cancer cells. Other theories have documented possible infection or a p53 gene resulting in conversion of the carcinoma to sarcoma [10,11].
Sarcomatoid carcinoma has many of the same presenting features as traditional colonic adenocarcinoma. Chief concerns on presentation range from rectal bleed [1,17,34] to abdominal pain , making it difficult to differentiate without tissue-proven diagnosis. Given the current literature and lack of overall treatment direction, many of these patients are being treated as cases of general colonic cancer. The major difference between sarcomatoid carcinoma and colonic adeno-carcinoma is the aggressive nature with poor prognosis of the tumor. As seen in our patient and numerous other patients in previous cases, the overall length of survival is short and aggressive therapy is needed to improve patient outcomes. Current therapy involves 5-fluorouracil, leucovorin, doxorubicin, and cisplatin, with additional radiation to treat patients in an adjuvant manner, but no evidence has shown their overall effect on the patient [16,20,28]. Additional research is necessary to determine appropriate chemotherapy regimens and prognostic factors. In addition, it has been noted that the metastasis of these tumors follows along the carcinoma origin, with spread to distant sites or lymph nodes rather than hematogenous spread as with sarcomas.
The rarity of sarcomatoid carcinoma leads to a difficult challenge when treating a newly diagnosed patient. Additional research needs to be performed on determination of optimal prognostic factors, role of adjuvant therapy, and appropriate surgical planning procedures.
In this current setting, the most appropriate management involves treating the patient along a colonic adenocarcinoma algorithm. In the end, due to the severity of the disease and rapid spread, it may be difficult to achieve appropriate studies to develop definitive treatment algorithms.
Carcinosarcoma is an extremely rare tumor, with little research guiding treatment guidelines. It has been identified as a biphasic tumor displaying origins from the epithelium and mesenchyme. Immunohistochemistry remains the standard for diagnosis. Current guidelines gathered from previous case reports suggest the treatment of choice for colorectal carcinosarcoma is the same as for adenocarcinoma. Additional research and prospective studies are needed to establish appropriate therapeutic guidelines and prognostic factors for carcinosarcoma.
FiguresFigure 1.. Original axial (A) and coronal (B) imaging highlighting associated inflammation, fluid collection and lymphadenopathy concerning for mass associated with cecum and ascending colon. Figure 2.. Expansion of previously identified collection concerning for mass associated with narrowing of ascending colon lumen in addition to wall thickening seen on axial (A) and coronal (B) computed tomography images. Figure 3.. Further expansion of previously identified mass with near obliteration of ascending colon lumen seen on axial (A) and coronal (B) imaging. Figure 4.. Associated lymphadenopathy after resection of previous right lower quadrant mass identified on axial (A) and coronal (B) imaging. Figure 5.. Pleomorphic nuclei admixed with spindle cells and anaplastic fiber nuclei (Hematoxylin and Eosin). Figure 6.. Pleomorphic nuclei, prominent nucleoli, and abundant mitotic figures adjacent to spindle cells and anaplastic fiber nuclei (hematoxylin and eosin). Figure 7.. Calretinin-positive staining highlighting nuclei and cytoplasm of tumor cells.
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