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Jaffe-Campanacci Syndrome

Multiple Non-Ossifying Fibromatosis


Presentation

  • The presentation, pathophysiology and management of the syndrome are briefly discussed.[ncbi.nlm.nih.gov]
  • She presented with multiple café-au-lait spots and multiple non-ossifying fibromata of the bones, noted on radiographs and proven by tissue biopsies. Only the left side was involved.[ncbi.nlm.nih.gov]
  • The case of a 15-year-old white boy with a clinical diagnosis of von Recklinghausen neurofibromatosis and a history of multiple fractures of long bones is presented.[ncbi.nlm.nih.gov]
  • In this article, we present a new syndrome mimicking JCS, which is characterized by a simple bone cyst and extraskeletal lesions.[ncbi.nlm.nih.gov]
  • Even more striking is a very rarely encountered lesion known as Jaffe-Campanacci syndrome, which also occurs in children who present with typical non-ossifying fibromatous tumors but in multiple sites.[ncbi.nlm.nih.gov]
Turkish
  • Castilian) Pronunciation Spanish (Castilian) Pronunciation Spanish (Spain) Pronunciation Spanish (Spain) Pronunciation Spanish (Spain) Pronunciation Spanish (Spain) Pronunciation Spanish (Mexico) Pronunciation Swedish Pronunciation Swedish Pronunciation Turkish[pronouncekiwi.com]
Leg Edema
  • Conclusion: To reduce her left leg edema, Z plasty on left ankle was done. After operation, her walking was better than before. To improve morphologic anomaly, upper eye lid plasty was done. GH trial could be helpful for her height growth.[abstracts.eurospe.org]
Coarctation of the Aorta
  • Coarctation of the aorta (isthmic portion) with a velocity of 2.3–2.4 m/sec, which did not require any intervention, was observed on the 2-dimensional echocardiogram.[e-sciencecentral.org]
Pallor
  • Persistent hyaloid vessels, lens dislocation, iris dysplasia, aniridia, coloboma, microphthalmia, ocular calcifications, and optic disc pallor have also been reported.[e-sciencecentral.org]
Chest Pain
  • Abstract Herein we report the clinical presentation and radiographic findings of a patient with a known history of multiple nonossifying fibromas, also known as the Jaffe-Campanacci syndrome, who presented with persistent pleuritic chest pain after a[ncbi.nlm.nih.gov]
  • pain and small pulmonary embolus after trauma in their case. 7 Kotzot et al also reported chylothorax and chylopericardium in their case with Jaffe-Campanacci's syndrome. 8 Our case had no thorax abnormality.[ncbi.nlm.nih.gov]
Aniridia
  • Persistent hyaloid vessels, lens dislocation, iris dysplasia, aniridia, coloboma, microphthalmia, ocular calcifications, and optic disc pallor have also been reported.[e-sciencecentral.org]
Sparse Hair
  • She had incomplete upper eyelids (coloboma with ectopic cilia on the left side and cryptophthalmos with dermolipoma on the right) and sparse hair with separated alopecia.[e-sciencecentral.org]
Subcutaneous Mass
  • The most characteristic scalp lesion in ECCL is nevus psiloliparus: a soft, subcutaneous mass with a demarcated area of alopecia. Dermal lesions of the face included multiple papular or polypoid nodules.[e-sciencecentral.org]

Workup

Pericardial Effusion
  • . , , Source MeSH Adult Aortic Valve Stenosis Biopsy Bone Neoplasms Chylothorax Female Fibroma Humans Male Pericardial Effusion Radiography Scoliosis Skin Diseases Syndrome Pub Type(s) Case Reports Journal Article Review Language eng PubMed ID 7894737[unboundmedicine.com]

Treatment

  • Treatment Options for this Tumor: Treatment of Jaffee Campanacci syndrome should consist of establishment of the diagnosis, if necessary, followed by an assessment of the known bone lesions.[bonetumor.org]
  • Make informed clinical choices for each patient, from diagnosis and treatment selection through post-treatment strategies and management of complications, with new evidence-based criteria throughout.[books.google.com]
  • Stay at the forefront of your field with updated treatment methods throughout, as well as an increased focus on patients with skin of color.[books.google.com]
  • Endovascular Treatment of a Ruptured Internal Thoracic Artery Pseudoaneurysm Presenting as a Massive Hemothorax in a Patient with Type I Neurofibromatosis.[connection.ebscohost.com]
  • Bone and soft tissue tumors: clinical features, imaging, pathology and treatment. 2nd edn New York: Springer,1999:85–90. [ Google Scholar ] 11. Holt JF, Wright EM. The radiologic features of neurofibromatosis.[ncbi.nlm.nih.gov]

Prognosis

  • Stabilization for risk of pathological fracture or actual pathological fracture is definitely indicated Outcomes of Treatment and Prognosis: As with most benign bone tumors, there have been reports of malignant transformation of non-ossifying fibromas[bonetumor.org]
  • […] knee, has a cloud-like, sunburst and codman's periosteal response, is worse if has a skip lesion on lung metastasis Classic Medullary Osteosarcoma tumor is more common in females, is 5-10 years older than in classic medullary ( 27), and has a better prognosis[memorize.com]
  • Outcome / Prognosis A fibrous cortical defect usually regresses with time. Prognosis is excellent even in patients who require curettage and bone grafting.[newhealthadvisor.com]
  • Treatment and prognosis Most NOFs require no treatment and no biopsy.[iranradiology.com]
  • Preferred Margin for this Tumor: marginal Outcomes of Treatment and Prognosis: After complete removal, recurrence is not expected and patients are expected to do well.[bonetumor.org]

Etiology

  • The etiology is discussed.[ncbi.nlm.nih.gov]
  • - A dictionary of medical eponyms Related people Henry Lewis Jaffe Mario Campanacci A syndrome of unknown etiology with variable expression characterized mainly by multiple non-ossifying fibromata, extraskeletal congenital anomalies such as café-au-lait[whonamedit.com]
  • They are organised into groups, and further divided into clinical, etiological or histopathological sub-types.[orpha.net]

Epidemiology

  • Review 2012 Review 2012 The evolving epidemiology of HIV/AIDS. Kevin M De Cock , Harold Jaffe , JamesW.[semanticscholar.org]
  • Author information 1 Clinical Genetics Branch, Division of Cancer Epidemiology and Genetics, National Cancer Institute, National Institutes of Health, Rockville, Maryland, USA. 2 1] Department of Human Genetics, University of Leuven, Leuven, Belgium [[ncbi.nlm.nih.gov]
  • Updated Molecular and Epidemiologic diagrams added to all new chapters.[books.google.es]
  • […] generation of clinical cancer genetics investigators.Hereditary Breast/Ovarian Cancer (HBOC) is based on a prospective cohort of 33 BRCA mutation-positive families with extensive clinical/epidemiologic data and biological samples.[grantome.com]
  • Epidemiology They are common in children and adolescents (peak incidence 10-15 year of age) and are usually not seen beyond the age of 30, as they spontaneously heal being gradually filled in by bone.[iranradiology.com]
Sex distribution
Age distribution

Pathophysiology

  • The presentation, pathophysiology and management of the syndrome are briefly discussed.[ncbi.nlm.nih.gov]
  • In pathophysiology of orthopaedic diseases. American Academy of Orthopaedic Surgeons, Rosemont, IL, pp 131–138 Google Scholar 41. Marks KE, Bauer TW (1989) Fibrous tumors of bone. Orthop Clin North Am 20:377–393 PubMed Google Scholar 42.[link.springer.com]
  • Pathophysiology, Evaluation, and Treatment ; This is an enhanced PDF from The Journal of Bone and Joint Surgery. 87:1848- 1864 ... Downloaded from ... tenstein and Jaffe in 19422. The true ....[wjicwiay.cf]

Prevention

  • Curettage and bone grafting promote healing of the lesions and should be considered to prevent pathological fracture.[ncbi.nlm.nih.gov]
  • The information presented here is not intended to diagnose, treat, cure or prevent any disease. Read disclaimer.[gulfdoctor.net]
  • Risk factors and prevention There are no particular risk factors for development of a solitary lesion. Treatment options The natural history of the lesion is spontaneous resolution with no symptoms.[orthopaedicsone.com]

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