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Recurrent Leiomyosarcoma With Malignant Transformation.

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Infantile fibrosarcoma (IFS) can recur and transform into high-grade sarcoma, necessitating careful multidisciplinary management. Early diagnosis and surgical planning are crucial for preventing recurrence and malignant transformation in childhood fibrosarcoma.

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Area of Science:

  • Pediatric Oncology
  • Surgical Pathology
  • Medical Oncology

Background:

  • Soft tissue sarcomas can originate anywhere, with fibrosarcoma being the most common non-rhabdomyosarcoma subtype in children.
  • Infantile fibrosarcoma (IFS), often congenital, presents in children under two, is locally aggressive but rarely metastatic, and typically affects the extremities.

Observation:

  • A 29-year-old presented with a fifth fibrosarcoma recurrence, which had transformed into high-grade leiomyosarcoma.
  • Initial diagnosis at two days old as lymphangioma, followed by multiple recurrences and transformations, highlighting diagnostic challenges.
  • Treatment involved multiple surgeries, neoadjuvant chemotherapy (doxorubicin, cisplatin), radiotherapy (IMRT), and antiangiogenesis inhibitors (bevacizumab), complicated by toxicity and recurrence.

Findings:

  • The case illustrates the potential for malignant transformation and aggressive recurrence of infantile fibrosarcoma over decades.
  • Despite aggressive multimodal treatment, including chemotherapy and radiotherapy, the tumor recurred, leading to severe complications and eventual patient death.
  • The patient experienced severe neuropathy and autotoxicity from chemotherapy, and hypertension from bevacizumab, limiting treatment options.

Implications:

  • A multidisciplinary approach is vital for managing IFS due to its high recurrence potential and risk of malignant transformation later in life.
  • Careful surgical planning and awareness of long-term recurrence risks are essential for improving outcomes in childhood fibrosarcoma.
  • This case underscores the need for vigilant long-term follow-up and tailored treatment strategies for patients with infantile fibrosarcoma.