Kanack Melissa, Collins Jessica, Fairbanks Timothy J, Gosman Amanda
From the *Division of Plastic Surgery, Department of Surgery, University of California San Diego; †Oasis MD; ‡Department of Surgery, University of California San Diego; §Rady Children's Hospital-San Diego; and ∥Division of Pediatric Surgery, Department of Surgery, University of California San Diego, San Diego, CA.
Ann Plast Surg. 2015 May;74 Suppl 1:S25-9. doi: 10.1097/SAP.0000000000000376.
Congenital-infantile fibrosarcoma (CIFS) is a rare pediatric soft tissue tumor that on initial clinical presentation can mimic an infantile hemangioma. Management of these conditions differs drastically, and delay in diagnosis and treatment may affect prognosis.
A 6-month-old male infant originally presented to dermatology for evaluation of a suspected right buttock hemangioma, present at birth as a small red mass just lateral to the gluteal cleft. The lesion rapidly grew over 2 weeks to a size of 4×4.5×4.5 cm, with violaceous color change and central ulceration. The patient received a course of cephalexin; however, the mass became painful with several episodes of bleeding and did not show clinical improvement. Magnetic resonance imaging and ultrasound supported the diagnosis of atypical hemangioma.
Plastic Surgery was consulted for excision due to recurrent bleeding. An excisional biopsy was undertaken and pathologic finding showed CIFS. The case was discussed with Hematology/Oncology and Pediatric Surgery, and the patient underwent re-excision and obtained clear margins. Initial metastatic workup was negative; however, patient had evidence of recurrent disease as well as pulmonary metastasis on his most recent surveillance imaging. He underwent re-excision of the gluteal mass and is currently receiving adjuvant chemotherapy.
This case demonstrates a rare malignancy initially misdiagnosed as a hemangioma. Consideration of CIFS is crucial in cases of complicated hemangiomas not responding to standard treatment. Treatment calls for excision with wide margins and/or adjuvant chemotherapy if excision is not possible. Classic pathologic findings, immunohistochemistry, and/or reverse transcription-polymerase chain reaction can confirm the diagnosis. A correct and rapid diagnosis of CIFS is critical to optimizing treatment and subsequent prognosis.
先天性婴儿纤维肉瘤（CIFS）是一种罕见的儿科软组织肿瘤，其最初临床表现可类似于婴儿血管瘤。这些疾病的治疗方法差异很大，诊断和治疗的延迟可能会影响预后。
一名6个月大的男婴最初因疑似右臀部血管瘤到皮肤科就诊，该血管瘤出生时为臀裂外侧的一个小红块。病变在2周内迅速生长至4×4.5×4.5厘米大小，伴有紫蓝色变色和中央溃疡。患者接受了头孢氨苄疗程治疗；然而，肿块变得疼痛，有几次出血，且未显示临床改善。磁共振成像和超声支持非典型血管瘤的诊断。
由于反复出血，咨询整形外科进行切除。进行了切除活检，病理结果显示为CIFS。与血液学/肿瘤学和小儿外科讨论了该病例，患者接受了再次切除并获得了切缘阴性。初始转移评估为阴性；然而，患者在最近的监测影像上有复发疾病以及肺转移的证据。他接受了臀肌肿块的再次切除，目前正在接受辅助化疗。
本病例显示了一种罕见的恶性肿瘤最初被误诊为血管瘤。对于对标准治疗无反应的复杂血管瘤病例，考虑CIFS至关重要。治疗需要广泛切缘切除和/或在无法切除时进行辅助化疗。经典病理结果、免疫组织化学和/或逆转录聚合酶链反应可确诊。CIFS的正确快速诊断对于优化治疗和后续预后至关重要。
