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Atomic Orbitals02:44

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An atomic orbital represents the three-dimensional regions in an atom where an electron has the highest probability to reside. The radial distribution function indicates the total probability of finding an electron within the thin shell at a distance r from the nucleus. The atomic orbitals have distinct shapes which are determined by l, the angular momentum quantum number. The orbitals are often drawn with a boundary surface, enclosing densest regions of the cloud.
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[Primary Orbital Liposarcoma].

Marina Guchlerner1, Susanne Pitz1

  • 1Orbitazentrum, Bürgerhospital und Clementine Kinderhospital gemeinnützige GmbH, Frankfurt am Main.

Klinische Monatsblatter Fur Augenheilkunde
|December 20, 2018
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Summary
This summary is machine-generated.

A rare orbital liposarcoma initially misdiagnosed as spindle cell lipoma presented as eye proptosis and pain. Surgical debulking and exenteration with radiotherapy were required for treatment.

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Area of Science:

  • Ophthalmology
  • Oncology
  • Genetics

Background:

  • Orbital masses can present with subtle symptoms like proptosis, potentially delaying diagnosis.
  • Spindle cell lipoma is a benign tumor, but differentiation from well-differentiated liposarcoma is critical.

Observation:

  • A 30-year-old male presented with a two-week history of painful left globe subluxation and a two-year history of painless proptosis.
  • Initial MRI revealed a retrobulbar mass, biopsied and diagnosed as spindle cell lipoma.
  • Surgical debulking via a transconjunctival approach was performed for proptosis relief.

Findings:

  • Repeated histology and immunohistochemistry confirmed a diagnosis of well-differentiated orbital liposarcoma.
  • The patient subsequently underwent orbital exenteration and adjuvant radiotherapy.
  • Genetic testing identified a heterozygote mutation in the ATM-gene on chromosome 11.

Implications:

  • This case highlights the importance of thorough histopathological and immunohistochemical analysis for accurate orbital tumor diagnosis.
  • The ATM gene mutation may have implications for understanding liposarcoma development and patient prognosis.
  • Multidisciplinary management, including surgery and radiotherapy, is crucial for treating orbital liposarcoma.