J Korean Med Sci.  2013 Jan;28(1):160-163. 10.3346/jkms.2013.28.1.160.

A Case of Congenital Infantile Fibrosarcoma of Sigmoid Colon Manifesting as Pneumoperitoneum in a Newborn

• Kim HY
• Cho YH
• Byun SY
• Park KH

• KMID: 2158017
• DOI: http://doi.org/10.3346/jkms.2013.28.1.160

Abstract

Congenital infantile fibrosarcoma (CIF) is a rare soft-tissue tumor in the pediatric age group and seldom involves the gastrointestinal tract. A 2-day-old boy was transferred to our hospital with a pneumpoperitoneum. After emergency operation, we could find a solid mass wrapping around a sigmoid colon and performed a segmental resection of sigmoid colon including a mass. Histopathologic examination showed an infantile fibrosarcoma origining from the muscular layer of colon. The baby was discharged on the 17th hospital day and followed for 1 yr without recurrence.

Keyword

Congenital Infantile Fibrosarcoma (CIF); Gastrointestinal

MeSH Terms

Colon, Sigmoid/pathology
Fibrosarcoma/congenital/*diagnosis/pathology
Humans
Infant, Newborn
Male
Peritoneum/radiography
Positron-Emission Tomography and Computed Tomography
Soft Tissue Neoplasms/congenital/*diagnosis/pathology

Figure

• 

  Fig. 1 Infantography shows a large amount of free-air in peritoneal cavity before operation.

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  Fig. 2 Grossly it shows a round mass wrapping around a sigmoid colon.

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  Fig. 3 A hypercellular and poorly circumscribed tumor mass in submucosa and muscularis proper (H&E stain, × 40), atypical spindle cells forming fascicular arrangement with mitoses and small amount of interstitial collagen in inlet (H&E stain, × 400).

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  Fig. 4 Immunohistochemical stains of tumor cells. Positive for vimentin and smmoth muscle actin (SMA, × 200) in A, Ki-67 proliferation index 20% in B, negative for desmin (desmin, × 200) and h-caldesmon (h-caldesmon, × 200) in C, D.

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  Fig. 5 Follow-up PET-CT shows no abnormal uptakes, evidence of tumor recurrence, 12 months later after operation.

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