Another such case is presented here who was successfully managed. Case presentation A 3-month-old Hindu baby boy presented with a congenital neck swelling. The apparent clinical diagnosis was lympho-venous malformation. [jmedicalcasereports.biomedcentral.com]

CIFS affects chiefly the distal portions of the extremities, but there are unusual presentations as lung, retroperitoneum and heart. [5],[6] In our presentation the tumor affects the proximal portion of the arm. [afrjpaedsurg.org]

A few days prior to presentation, the mass rapidly increased in size, with visible blood vessels that bled upon touch. At presentation the child was pale, hypoactive, and had profuse oozing of blood and purulent discharge from the mass. [ncbi.nlm.nih.gov]

Indian J Surg Oncol 5:297–299 Article PubMed Google Scholar Brohi SR, Dilber M (2012) Congenital osteolytic fibrosarcoma of dura presenting as scalp mass. [link.springer.com]

A seven-month-old female patient presented with a history of a congenital, violaceous, fast-growing lesion located on the right plantar surface. [scielo.br]

• Soft Tissue Mass

  Rapidly progressive shoulder soft tissue mass in an 8 week old girl. Clin Orthop Relat Res 2011; 469: 624–629. [nature.com]

  Soft tissues tumors account for 7% of the malignant tumors in childhood and fibro- and myoblastic tumors account for 12% of the soft tissues tumors in childhood. [thefetus.net]

  MRI On MRI infantile fibrosarcomas are heterogeneous soft tissue masses and can show well-defined or infiltrative tumor margins. They might show low signal intensity fibrous streaks 2. [radiopaedia.org]

  Large heterogeneously enhancing soft tissue mass measuring 8×3 cm and showing areas of enhancement and necrosis. The second patient was a 2-month-old full-term boy born to first-degree consanguineous parents. [ncbi.nlm.nih.gov]

• Anemia

  […] fetalis Anemia, TCP None Died at day 3 of life 1999 [10] 1 Birth Arm Anemia, TCP S* NED at 6 y 1999 [11] 1 27 days Forearm Anemia S NED at 2.5y 2003 [12] 1 Birth Hand Anemia CT NED at 30 m 2004 [13] 1 Birth Palm Anemia, TCP, prolonged PT, PTT, low fibrinogen [ncbi.nlm.nih.gov]

  The child developed severe anemia (hemoglobin of 4.4g/dL), requiring a blood transfusion. The platelet count was normal. Histopathology was suggestive of kaposiform hemangioendothelioma. [scielo.br]

  Prenatal diagnosis is possible from 26 weeks of gestation by ultrasound and magnetic resonance imaging (RMI). [8] At birth, the tumor is sometimes confused with hemangioma and can cause fetal anemia and requires transfusion as in our patient. [8] Our [afrjpaedsurg.org]

  However, a similar phenomenon to Kasabach-Merritt syndrome (hemolytic anemia, thrombocytopenia, and secondary consumptive coagulopathy) which occurred in other vascular tumors could be the basis given the high vascularity of CIF. [frontiersin.org]

• Purpura

  Capillary hemangioma with extensive purpura. Am J Dis Child. 1940;59:1063–70. [Google Scholar] 17. Scheier M, Ramoni A, Alge A, et al. Congenital fibrosarcoma as cause for fetal anemia: prenatal diagnosis and in utero treatment. [ncbi.nlm.nih.gov]

• Hydrops Fetalis

  The 4 patients who presented with hydrops fetalis died shortly after birth and 2 other patients died of bleeding complications. [ncbi.nlm.nih.gov]

• Scalp Mass

  Indian J Surg Oncol 5:297–299 Article PubMed Google Scholar Brohi SR, Dilber M (2012) Congenital osteolytic fibrosarcoma of dura presenting as scalp mass. [link.springer.com]

• Suggestibility

  A frozen section sent during excision was suggestive of malignancy. Complete gross resection of the lesion was done. There were no obviously enlarged neck nodes. [jmedicalcasereports.biomedcentral.com]

  Histopathology was suggestive of kaposiform hemangioendothelioma. Treatment with oral prednisolone (2mg/kg/day) was started but was interrupted after one month, due to lack of a response (Fig. 1B). Figure 1 (A) Ulcerated and bleeding tumor mass. [scielo.br]

  The stage of a tumor suggests which form of treatment is most appropriate, and gives some indication of prognosis. A fibrosarcoma may be localized, meaning it has not spread beyond the bone where it arose. [childrenshospital.org]

  Recurrent EML4-NTRK3 fusions in infantile fibrosarcoma and congenital mesoblastic nephroma suggest a revised testing strategy. [pubmed.ncbi.nlm.nih.gov]

  The cancer stage suggests which form of treatment is most appropriate and gives some indication of prognosis. A fibrosarcoma may be localized, meaning it has not spread beyond the bone where it arose or beyond nearby tissues. [dana-farber.org]

• Aggressive Behavior

  Axial tumors (15 cases, 29%) had a local recurrence rate of 33%, which is similar to that for extremity lesions, but the metatstatic and mortality rates were 26% each indicating a more aggressive behavior for axial lesions. [pubmed.ncbi.nlm.nih.gov]

A metastatic workup was negative. No chemoradiotherapy was initiated and the child was kept under close follow-up. A follow-up contrast-enhanced computed tomography scan (CECT) of his neck and chest showed no residue or recurrence at 3 and 6 months. [jmedicalcasereports.biomedcentral.com]

Childhood Fibrosarcoma Treatment Team The Dana-Farber/Boston Children's solid tumor treatment team includes childhood soft tissue tumor specialists who care for children with fibrosarcoma. [dana-farber.org]

MRI performed at the end of treatment, 24 and 30 months off treatment revealed no residual lesion. Currently she is almost 3 years off treatment and has remained well. She has full function of the right limb. Figure 2. [frontiersin.org]

Discussion Approaches to treatment of CIF may vary from simple observation to surgical intervention, chemotherapy, or both. [ncbi.nlm.nih.gov]

What are the treatment options for fibrosarcoma? Fibrosarcoma treatments have improved dramatically in recent years, particularly for patients with localized disease. [childrenshospital.org]

Prognosis: Although these tumors display histological features of malignancy and frequently recur, they have a relatively good prognosis and only rarely metastasize. Better if t(12 ;15) (p13 ; q25 ) translocation is found. [thefetus.net]

The stage of a tumor suggests which form of treatment is most appropriate, and gives some indication of prognosis. A fibrosarcoma may be localized, meaning it has not spread beyond the bone where it arose. [childrenshospital.org]

The cancer stage suggests which form of treatment is most appropriate and gives some indication of prognosis. A fibrosarcoma may be localized, meaning it has not spread beyond the bone where it arose or beyond nearby tissues. [dana-farber.org]

The prognosis of this tumor is relatively good compared to adult forms. We report an unusual case of CIFS occurring in new-born mimicking an hemangioma and causing hemorrhage in the neonatal period. [afrjpaedsurg.org]

The infantile variant shares histopathological characteristics with adult fibrosarcoma but has a better prognosis. [scielo.br]

Etiology: For many years, it has been the hope, expressed in many epidemiological studies, to identify the cause or causes of malignancies that occur in early life. [thefetus.net]

Infantile fibrosarcomas (CIFS) are soft tissue sarcomas that are diagnosed at or soon after birth. [2] Its etiology remains unknown. [afrjpaedsurg.org]

Etiology: For many years, it has been the hope, expressed in many epidemiological studies, to identify the cause or causes of malignancies that occur in early life. [thefetus.net]

A similar pathophysiology is likely to be responsible for the findings in CIF, due to the known high vascularity of this tumor. One of our patients (patient #2) developed life-threatening hepatopathy as a complication of VAC chemotherapy. [ncbi.nlm.nih.gov]

The pathophysiology of consumptive coagulopathy in CIF is not well-understood. [frontiersin.org]

Whenever feasible early surgery should be performed in very young patients with large CIF at risk of life threatening bleeding to prevent mortality. [frontiersin.org]

Being able to anticipate these side effects can help the care team, child, and family prepare (and, in some cases, prevent) these complications from occurring, if at all possible. [childrenshospital.org]

After treatment, your child may receive rehabilitative and supportive care to help them prevent or treat side effects and get back to functioning in their daily lives. [dana-farber.org]

In our patient, we opted for primary chemotherapy to prevent metastasis, especially after finding a rapid increase in tumor volume in a few days. We decided to perform an amputation of the upper limb. [afrjpaedsurg.org]

Prevention How can I reduce my risk for fibrosarcoma? You can reduce your risk for fibrosarcoma by understanding your family medical history. Fibrosarcoma appears to be related to some inherited conditions. [my.clevelandclinic.org]