Características clínicas y radiológicas de una cohorte de niños con trombosis venosa intracraneal

Abstract

Introduction: Fibrous hamartoma of infancy (FHI) is a benign, soft tissue tumor that usually occurs in children and has a characteristic histological morphology. Objective: To describe a case of congenital FHI with atypical histological and clinical characteristics. Clinical case: Full-term male newborn, with no perinatal morbid history was referred to dermatology due to a congenital erythematous plaque in the umbilical region. The histological study showed a fusocelullar proliferation in dermis and hypodermis of biphasic distribution, with an infiltrative, swirling pattern and bundles of spindle fibroblast-like and myofibroblast-like cells, associated in depth with a mature adipose tissue component. The immunohistochemical study revealed diffuse positivity for CD34, and focal positivity for FXIIIa, without immunoreactivity for actin, desmin, MyoD1, S100, HMB45, Melan-A, or EMA. Fluorescent in situ hybridization (FISH) was negative for platelet-derived growth factor receptor beta (PDGFR-beta) and for ETV6 gene. PDGFR-beta and ETV6 gene are present in congenital dermatofibrosarcoma protuberans and infantile fibrosarcoma, respectively. This history, in addition to previous histological findings, supported the diagnosis of FHI. Surgical resection was performed, without signs of recurrence during clinical follow-up. Conclusion: It is important to consider the FHI within the differential diagnosis of subcutaneous tumors in children, especially in those under two years of age. Although its behavior is benign, it is similar to multiple benign and malignant lesions, which makes it imperative to perform a histological study in front of suspicious clinical lesions.

Clinical characteristics, anticoagulation therapy, and short-term outcomes of neonatal cerebral sinovenous thrombosis: a cross-sectional study from a Chilean center

“…This may be due to the lower risk of intracranial hypertension due to hemorrhage and venous infarction in younger children, resulting in a lower risk of mortality. In fact, compared with the same base population, non-neonates had a higher risk of parenchymal lesions than our neonates [23].…”

Cerebral venous thrombosis in Latin America: A critical review of risk factors, clinical and radiological characteristics

“…The main epidemiological findings in each Latin American country are described in the Supplementary Table . Only 9 Latin American countries were represented (9/36, 27.3%): Argentina ( 12 , 18 , 29 , 30 ), Brazil ( 8 , 15 , 19 , 20 , 22 , 25 , 26 ), Chile ( 9 , 23 , 24 ), Colombia ( 13 , 31 , 33 ), Costa Rica ( 28 , 32 ), Cuba ( 14 ), Guadeloupe ( 17 ), Mexico ( 11 , 16 , 21 , 27 ), and Uruguay ( 10 ). In Figure 2A , it is possible to identify the reported CVT cases in large series in Latin America.…”

“…Futhermore, an important limitation is that there were no age and sex restrictions on study participants in our selection of articles. In this context, two articles in our sample ( 23 , 32 ) included only pediatric patients, while one article ( 21 ) only addressed puerperal women, although this did not significantly affect our epidemiological representation. In this review, the unavailability of data (such as mean age, detailed risk factors, or imaging features) was another limiting aspect.…”

Cerebral Venous Thrombosis in Pediatric Age: Risk Factors and Prognosis

“…In 6 patients, major sequelae were detected at follow-up, namely epilepsy (n=3), sensor-motor deficits (n=2) and cognitive impairment (n=3). Median age at time of cognitive assessment was 6 years old (2)(3)(4)(5)(6)(7)(8)(9)(10)(11)(12)(13)(14)(15)(16).…”

“…[2][3][4][5][6][7][8][9][10][11][12][13][14][15][16] Median time of follow-up was 5.8 years (1.1-13.3 years). No recurrence of CVT was registered.…”