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Year : 2014  |  Volume : 57  |  Issue : 3  |  Page : 504-505
Calcifying fibrous tumor of the rectum: A case report

1 Department of Pathology, Dankook University College of Medicine, Cheonan, Korea
2 Department of Internal Medicine, Dankook University College of Medicine, Cheonan, Korea
3 Department of Biomedical Engineering, Dankook University College of Medicine, Cheonan, Korea

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Date of Web Publication14-Aug-2014

How to cite this article:
Lee W, Jeon SM, Lee Sh. Calcifying fibrous tumor of the rectum: A case report. Indian J Pathol Microbiol 2014;57:504-5

How to cite this URL:
Lee W, Jeon SM, Lee Sh. Calcifying fibrous tumor of the rectum: A case report. Indian J Pathol Microbiol [serial online] 2014 [cited 2020 Jun 5];57:504-5. Available from: http://www.ijpmonline.org/text.asp?2014/57/3/504/138806


A 46-year-old woman was referred to our institution upon having a tumor detected incidentally by screening colonoscopy. A repeat colonoscopy revealed a submucosal mass with intact overlying mucosa in the rectum, and endoscopic ultrasound (EUS) at this time demonstrated a well-defined homogeneously hypoechoic solid mass measuring 9 mm in its largest diameter. No calcification was detected by EUS. These findings created suspicion for a submucosal tumor such as a gastrointestinal stromal tumor (GIST), leiomyoma, or carcinoid tumor. The mass was removed by endoscopic submucosal dissection. The sectioned surface of the mass was white and firm. Microscopic examination of whole mount serial sections revealed a well circumscribed but nonencapsulated tumor in the submucosa [Figure 1]a. The tumor was composed of paucicellular bland spindle cells with densely hyalinized stroma. Lymphocytes infiltrated with occasional germinal center formation [Figure 1]b and abundant plasma cells infiltrated in sclerotic stroma [Figure 1]c. A peritumoral lymphoid cuff was focally noted. A small psammomatous calcification was detected within sclerotic stroma [Figure 1]a. Immunohistochemically, the spindle cells were positive for vimentin and factor XIIIa and negative for CD34, c-kit, S-100 protein, smooth muscle actin (SMA), bcl-2, CD99 and anaplastic lymphoma kinase. A significant number of IgG4-positive plasma cells was noted [Figure 1]d. The number of IgG4-positive plasma cells was up to 15/hpf. IgG4-positive plasma cells amongst all the plasma cells accounted for 5-10%. Serum IgG4 level was not evaluated. The patient was not known to have an IgG4-related sclerosing disease.
Figure 1: (a) A whole mount view shows a well circumscribed sclerotic submucosal tumor. A small psammomatous calcification (arrow and inset) is seen (H and E, ×12.5). (b) Lymphocytes infiltrate with
germinal centers (H and E, ×100). (c) A few bland-looking spindle cells and many plasma cells are noted in sclerotic stroma (H and E, ×400). (d) Immunohistochemically, considerable IgG4-positive plasma cells are noted (immunohistochemistry, ×400)

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Calcifying fibrous tumor (CFT) is a rare benign mesenchymal tumor composed of paucicellular bland spindle cells, lymphoplasma cell infiltrates, and abundant hyalinized collagenous stroma with small calcifications. CFT occurs at various sites such as extremities, trunk, neck, scrotum, pleura, mediastinum and peritoneum. Gastrointestinal CFT is extremely rare. So far about 30 cases of gastrointestinal CFTs have been reported. [1] In the gastrointestinal tract, the stomach is the most common site, followed by the small intestines. [1] To the best of our knowledge, only two colorectal CFTs have been reported in the literature. [2],[3] In contrast to visceral CFT, gastrointestinal CFT are frequently minute, solitary and incidental lesions and have a benign course with a lack of recurrence. [1] As such, conservative management is warranted for gastrointestinal CFT. [1]

Gastrointestinal CFT arises in subserosal, intramural and/or submucosal layers. Endoscopically, it is difficult to distinguish gastrointestinal CFT from other submucosal tumors of the gastrointestinal tract. Gastrointestinal CFT must be distinguished histologically from various mesenchymal tumors such as the GIST, the inflammatory myofibroblastic tumor (IMT), the solitary fibrous tumor and the reactive nodular fibrous pseudotumor.

The pathogenesis of CFT remains unknown. It has been hypothesized that CFT is the sclerosing end stage of IMT; however, immunohistochemical, electron microscopic and cytogenetic studies have allowed further differentiation between these two entities. [4] Immunohistochemically, CFT is diffusely positive for factor XIIIa and negative for SMA; however, IMT is focally positive for factor XIIIa and diffusely for SMA. [4] Electron microscopically, the spindle cells of CFT are consistent with immature fibroblasts; however, those of IMF->IMT correspond to fibroblasts and myofibroblasts. [4] In the same context, the present case was found to have a homogeneous histology without any transitional areas to IMT. This finding suggests that CFT is a disease entity completely distinct from IMT.

More recently, a novel theory of CFT as an IgG4-related disease has been proposed for the pathogenesis of CFT. Sclerosing angiomatoid nodular transformation (SANT) is a rare splenic vascular lesion with stromal sclerosis and plasma cell infiltrate. Cases of concomitant abdominal CFT and SANT have been reported, mainly out of Taiwan. [5] In those cases, a significant number of IgG4-positive plasma cells was found in both CFT and SANT tissue samples, suggesting a relationship to IgG4-related process. According to the report by Kuo et al., [5] 5 out of 10 patients with SANT had single or multiple CFTs in the abdominal cavity. In a study of gastric CFTs unrelated to SANT, 5 out of 7 cases contained scattered IgG4-positive plasma cells in CFT lesions. [1] Although the present case was a very small CFT lesion, the characteristic sclerosing histology as well as the significant number of IgG4-positive plasma cells supported that CFT might be an IgG4-related sclerosing disease.

Rectal CFT is extremely rare but it should be considered in the differential diagnosis of submucosal tumor of the rectum.

   Acknowledgment Top

This research was conducted by the research fund of Dankook University in 2012.

   References Top

Agaimy A, Bihl MP, Tornillo L, Wünsch PH, Hartmann A, Michal M. Calcifying fibrous tumor of the stomach: Clinicopathologic and molecular study of seven cases with literature review and reappraisal of histogenesis. Am J Surg Pathol 2010;34:271-8.  Back to cited text no. 1
Shi Q, Xu MD, Zhong YS, Zhou PH, Wu HF, Yao LQ. The laparoscopic-endoscopic cooperative surgery for the colonic calcifying fibrous tumor: One case report. J Laparoendosc Adv Surg Tech A 2012;22:996-8.  Back to cited text no. 2
Im S, Jung JH, Yoo C, Choi HJ, Yoo J, Kang CS. Calcifying fibrous tumor presenting as rectal submucosal tumor: First case reported in rectum. World J Surg Oncol 2014;12:28.  Back to cited text no. 3
Hill KA, Gonzalez-Crussi F, Chou PM. Calcifying fibrous pseudotumor versus inflammatory myofibroblastic tumor: A histological and immunohistochemical comparison. Mod Pathol 2001;14:784-90.  Back to cited text no. 4
Kuo TT, Chen TC, Lee LY. Sclerosing angiomatoid nodular transformation of the spleen (SANT): Clinicopathological study of 10 cases with or without abdominal disseminated calcifying fibrous tumors, and the presence of a significant number of IgG4+ plasma cells. Pathol Int 2009;59:844-50.  Back to cited text no. 5

Correspondence Address:
Wonae Lee
Department of Pathology, Dankook University College of Medicine, 16-5 Anseo-Dong, Cheonan Chungcheong Nam Do 330-715
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0377-4929.138806

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