Presentation
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]
Entire Body System
- 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]
- Swelling
Initially a small midline swelling, it progressively increased in size with age. It was soft and compressible with an overlying bluish hue at places. [jmedicalcasereports.biomedcentral.com]
All were males and presented with scalp swelling at birth which grew rapidly in size. The tumor was located in the left temporal region in two cases and the right temporoparietal region in one case. [link.springer.com]
Unusual swelling. This might be a sign your tumor is pressing on your blood vessels. Unintended weight loss. What causes fibrosarcoma? Researchers haven’t pinpointed exactly what causes fibrosarcoma. [my.clevelandclinic.org]
There was no lymph node swelling in the right upper arm, cubital fossa, or axilla. [frontiersin.org]
- Surgical Procedure
Of note, 6 of the 14 surgical procedures consisted of amputation. Table 1 Summary of reported cases of CIF associated with bleeding diathesis at diagnosis. [ncbi.nlm.nih.gov]
Gastrointestinal
- Vomiting
Persistent nausea and vomiting. What questions should I ask my doctor? Fibrosarcoma is a rare form of cancer. You might have questions about a cancer that few people know about. [my.clevelandclinic.org]
- Nausea
Persistent nausea and vomiting. What questions should I ask my doctor? Fibrosarcoma is a rare form of cancer. You might have questions about a cancer that few people know about. [my.clevelandclinic.org]
Psychiatrical
- 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]
Workup
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]
Treatment
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
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
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]
Epidemiology
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]
Pathophysiology
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]
Prevention
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]