Tuberous sclerosis complex
J. K. Cowell in Molecular Genetics of Cancer, 2003
Cardiac rhabdomyoma is often the earliest detectable sign of TSC since it can be visualized by fetal ultrasound scanning during pregnancy. Cardiac insufficiency, due to the presence of cardiac rhabdomyomas, increases the chance of perinatal and postnatal mortality in TSC. However, since follow-up ultrasound imaging studies show that these tumors usually become smaller during postnatal development, and sometimes even disappear completely, the chance of cardiac insufficiency in later life is reduced. The cause of this age-related regression is unknown. The reported prevalence of cardiac rhabdomyoma in TSC patients varies between 30% (Gomez, 1988) and 47% (Jozwiak et al., 1994).
Tumors of Heart
P. Chopra, R. Ray, A. Saxena in Illustrated Textbook of Cardiovascular Pathology, 2013
The cells of rhabdomyoma are quite characteristic. They are large with abundant clear cytoplasm in which slender cytoplasmic strands extend from the nucleus to the cell wall. Latter have the appearance of "spider" cells. These cells possess abundant glycogen which is well brought out with periodic acid-Schiff stain. They also show positive reaction with antisera to myoglobin, desmin and actin (Fig. 12.27c). Ultrastructural features include abundant glycogen, irregular myofilaments with Z bands and other organelles. Large and single rhabdomyomas can be subject to surgery with good long-term results.
Rhabdomyosarcoma
Dongyou Liu in Tumors and Cancers, 2017
Skeletal muscle tumors are subdivided into benign (rhabdomyoma [adult type, fetal type, and genital type]) and malignant (embryonal rhabdomyosarcoma [RMS], alveolar RMS, spindle cell or sclerosing RMS, and anaplastic or pleomorphic RMS) [1].
Triton tumor of the orbit
Published in Orbit, 2020
Atanu Barh, Bipasha Mukherjee, Kirthi Koka, Subramanian Krishnakumar
Peripheral nerve sheath tumors are derived from the pluripotent Schwann cells of the neurilemmal sheath.Many unusual histologic variants within an MPNST have been described, including osteoid, benign striated muscle, cartilage, nevus cells, malignant melanoma, malignant neuroepithelium, liposarcoma, angiosarcoma, and rhabdomyosarcoma (embryonal and pleomorphic).6 The exact pathogenesis of the rhabdomyosarcomatous differentiation within the substance of a peripheral nerve tumor is unclear. MTT was first described by Mason in 1932 as “nerve rhabdomyoma.” In 1973, Woodruff coined the name “malignant triton tumor” inspired by Locatelli’s experiment on triton salamanders capable of growing muscle and bone from nerve tissue.6 Locatelli proposed that endoneurial cells of “neuromas” may differentiate into muscle tissue under the organizing influence of motor nerve fibers. However, limb and muscle regeneration in tritons is not dependent upon motor nerve innervation as even aneurogenic forelimbs of young salamanders may regenerate.6 Later, Masson proposed the more acceptable theory that neoplastic Schwann cells can transform into striated muscle elements which can give rise to rhabdomyosarcomatous differentiation.6
Surgical management of primary parapharyngeal space tumors in 103 patients at a single institution
Published in Acta Oto-Laryngologica, 2018
Fenglin Sun, Yan Yan, Dongmin Wei, Wenming Li, Shengda Cao, Dayu Liu, Guojun Li, Xinliang Pan, Dapeng Lei
Among the benign tumor group of 90 patients, the patients did not receive any further treatment postoperatively. Six patients were lost to follow-up and 84 patients were followed-up for 31–84 months with no recurrence. In contrast, for malignant tumors, postoperative radiotherapy was applied in four patients with lymphoma and combined chemo-radiotherapy was given to five patients, including two with leiomyosarcoma, one with myxofibrosarcoma, one with liposarcoma, and one with malignant rhabdomyoma. The auxiliary treatment was unknown in one patient with clear cell carcinoma. The remaining three patients in the malignant tumor group refused any postoperative treatment. Thirteen patients with malignant tumors were followed-up for 8–51 months. Seven patients died, of which three patients died of recurrence of leiomyosarcoma, myxofibrosarcoma, or liposarcoma. Four patients died of metastasis, including one with malignant rhabdomyoma and three with lymphoma. Two patients with a malignant tumor were lost to follow-up.
Related Knowledge Centers
- Striated Muscle Tissue
- Neoplasm
- Heart
- Vagina
- Malignancy
- Tongue
- Tuberous Sclerosis
- Rhabdomyosarcoma
- Fetal Rhabdomyoma
- Ectomesenchymal Chondromyxoid Tumor