BACKGROUND: Large congenital neoplasms of the extremities may be associated with coagulopathies and significant hemorrhage in the neonatal period. At times, the differences between coagulation derangements can be very subtle, leading to errors in diagnosis. Infants with vascular lesions and coagulopathies are often found to have the Kasabach-Merritt phenomenon, which is a platelet-trapping coagulopathy. However, other neoplasms or vascular malformations can be accompanied by disseminated intravascular coagulation. It is important to obtain accurate diagnoses of the neoplasm and the coagulopathy because the treatments of similar-appearing tumors and coagulopathies can be markedly different. METHODS: The authors report the case of a newborn with a congenital tumor of the left hand that was accompanied by a coagulopathy that caused significant bleeding. RESULTS: A presumption was made by the neonatal critical care physicians and hematologists that the infant had a kaposiform hemangioendothelioma along with the Kasabach-Merritt phenomenon. However, steroid treatment did not reduce the size of the mass or correct the coagulopathy. Only after obtaining consultation with a hand surgeon and a tissue diagnosis was it learned that the patient had an infantile fibrosarcoma that was accompanied by disseminated intravascular coagulation. Limb-sparing resection of the lesion along with chemotherapy markedly improved the patient's condition. CONCLUSIONS: Large congenital neoplasms presenting with attendant bleeding diatheses must be rapidly and accurately diagnosed with both a biopsy-proven tissue diagnosis and a hematologic characterization of the nature of the coagulopathy. The differential diagnosis of a vascular-appearing mass in the extremity can be subtle, and presumptive diagnosis, as occurred in this case, can lead to incorrect or delayed treatment. Specifically, kaposiform hemangioendothelioma must be differentiated from infantile fibrosarcoma. The principles of infantile fibrosarcoma treatment are limb-sparing resection and chemotherapy.
BACKGROUND: Large congenital neoplasms of the extremities may be associated with coagulopathies and significant hemorrhage in the neonatal period. At times, the differences between coagulation derangements can be very subtle, leading to errors in diagnosis. Infants with vascular lesions and coagulopathies are often found to have the Kasabach-Merritt phenomenon, which is a platelet-trapping coagulopathy. However, other neoplasms or vascular malformations can be accompanied by disseminated intravascular coagulation. It is important to obtain accurate diagnoses of the neoplasm and the coagulopathy because the treatments of similar-appearing tumors and coagulopathies can be markedly different. METHODS: The authors report the case of a newborn with a congenital tumor of the left hand that was accompanied by a coagulopathy that caused significant bleeding. RESULTS: A presumption was made by the neonatal critical care physicians and hematologists that the infant had a kaposiform hemangioendothelioma along with the Kasabach-Merritt phenomenon. However, steroid treatment did not reduce the size of the mass or correct the coagulopathy. Only after obtaining consultation with a hand surgeon and a tissue diagnosis was it learned that the patient had an infantile fibrosarcoma that was accompanied by disseminated intravascular coagulation. Limb-sparing resection of the lesion along with chemotherapy markedly improved the patient's condition. CONCLUSIONS: Large congenital neoplasms presenting with attendant bleeding diatheses must be rapidly and accurately diagnosed with both a biopsy-proven tissue diagnosis and a hematologic characterization of the nature of the coagulopathy. The differential diagnosis of a vascular-appearing mass in the extremity can be subtle, and presumptive diagnosis, as occurred in this case, can lead to incorrect or delayed treatment. Specifically, kaposiform hemangioendothelioma must be differentiated from infantile fibrosarcoma. The principles of infantile fibrosarcoma treatment are limb-sparing resection and chemotherapy.
Authors: Mayssaa Salman; Nabil J Khoury; Ibrahim Khalifeh; Hussein A Abbas; Marianne Majdalani; Miguel Abboud; Samar Muwakkit; Hassan El Solh; Raya Saab Journal: Am J Case Rep Date: 2013-11-15