Solitary infantile myofibromatosis in the femur

T Yamamoto1, K Mizuno, K Hanioka

  • 1Departments of Orthopedic Surgery and; Pathology, Kobe University, Kobe, Japan. yamatetu@med.kobe-u.ac.jp

Insights

This case study details a rare solitary infantile myofibromatosis in an infant's femur. The benign tumor presented as an osteolytic lesion, confirmed by pathological and immunohistochemical analysis.

Area of Science:

  • Pediatric Oncology
  • Skeletal Pathology
  • Soft Tissue Tumors

Background:

  • Infantile myofibromatosis is a rare benign tumor affecting infants.
  • The solitary type typically presents as a single lesion.
  • Long bone involvement is uncommon, especially in the diaphyseal region.

Observation:

  • A case of solitary infantile myofibromatosis involving the right femur in an 11-month-old female is presented.
  • Radiographic imaging revealed a well-defined osteolytic lesion in the femoral diaphysis.
  • Pathological examination showed spindle-shaped cells with eosinophilic cytoplasm, fascicular arrangement, and cleft-shaped vascular spaces.

Findings:

  • Immunohistochemical staining confirmed tumor cell reactivity for vimentin, alpha-smooth muscle actin, and desmin.
  • Ultrastructural analysis identified numerous thin and intermediate filaments within the tumor cell cytoplasm.
  • This represents the third reported instance of solitary infantile myofibromatosis specifically affecting long bones.

Implications:

  • This case contributes to the limited literature on infantile myofibromatosis of long bones.
  • Accurate diagnosis through imaging, pathology, and immunohistochemistry is crucial for appropriate management.
  • Understanding the cellular and ultrastructural characteristics aids in differentiating this benign entity from other bone lesions.

Related Concept Videos

Classification of Skeletal Muscle Fibers01:48

Classification of Skeletal Muscle Fibers

Skeletal muscles continuously produce ATP to provide the energy that enables muscle contractions. Skeletal muscle fibers can be categorized into three types based on differences in their contraction speed and how they produce ATP, as well as physical differences related to these factors. Most human muscles contain all three muscle fiber types, albeit in varying proportions.
Slow-Twitch Muscle Fibers
Slow oxidative, muscle fibers appear red due to large numbers of capillaries and high levels of...
Formation of Muscle Fibers from Myoblasts01:13

Formation of Muscle Fibers from Myoblasts

De novo myogenesis, or the formation of muscle fibers, begins during the early embryonic stages. The skeletal muscle is formed from somites– blocks of embryonic cell layers. The somites are further divided into dermatomes, myotomes, sclerotomes, and syndetomes. Among these, the myotomes give rise to muscle fibers.
Muscle progenitor cells (MPCs) are formed from the myotomes. MPCs express genes that encode the transcription factors Pax3 and Pax7. Along with Pax 3/7, other transcription factors...
Satellite Stem Cells and Muscular Dystrophy01:21

Satellite Stem Cells and Muscular Dystrophy

Satellite stem cells or myosatellite cells are quiescent stem cells that Alexander Mauro first identified in 1961. These cells are located between the sarcolemma, the plasma membrane of muscle fibers, and the basal lamina, the connective tissue sheath covering it. These mononucleated cells are activated in response to muscle injury, can transform into myoblasts, and may form or repair muscle fibers. Myosatellite cells can provide additional myonuclei for muscle regeneration or return to a...
Bones of the Lower Limb: Femur and Patella01:16

Bones of the Lower Limb: Femur and Patella

The femur is the body's longest and strongest bone spanning the thigh region. Its head articulates with the acetabulum of the hip bone to form the hip joint. A minor indentation on the medial side of the femoral head, called the fovea capitis, serves as the site of attachment for the ligament of the head of the femur. This weak ligament spans the femur and acetabulum and supports the hip joint. The narrowed region below the head is the neck of the femur. The inclination angle between the neck...