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Photomicrograph showing a specimen of tissue taken from the lipomyelomeningocele 1 day after birth. It demonstrates connective tissue containing striated muscle fibers without signs of malignancy (HE). b Photomicrograph of tissue taken at the second operation, showing a biopsy specimen of the malignant mesenchymal tumor with polymorphic and hyperchromatic nuclei and mitotic figures. Desmin and myogenin stains (not shown) displayed a strong immunoreactivity of tumor cells. The findings were characteristic of an embryonal rhabdomyosarcoma (HE; courtesy of Dr. J. Bohl and Dr. S. Doostkam, Institute of Neuropathology, University of Mainz).
Source publication
Malignant tumors arising within dysrhaphic malformations are very rare and are mostly teratomas; so far, only one rhabdomyosarcoma has been reported in this context. We report another case of a girl with lipomyelomeningocele who developed a lumbar rhabdomyosarcoma 2 years after birth and primary closure of the neural tube defect. We present clinica...
Context in source publication
Context 1
... tumor with features typical of embryonal rhabdomyosarcoma. The compar- ison with histological specimens of subcutaneous fat tissue taken at the primary closure of the lipomyelomeningocele showed unusual bundles of skeletal muscle fibers at that time, possibly being the ori- gin of the rhabdomyosarcoma, but with no tumor characteristics ( fig. ...
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Citations
... RMS has also been reported to occur as a congenital tumor. Congenital dysrhaphic malformation of the spine may predispose children to intra-lesional development of RMS [7]. ...
... In the present case, posterior paresis, the predominant symptom, was believed to be caused by rhabdomyosarcoma invading the thoracic spinal canal, compressing the ninth to eleventh thoracic vertebrae. Rhabdomyosarcoma is a highly invasive malignant tumor and invasion of the thoracic cord has previously been reported in humans and cats [3,8]. To the best of our knowledge, there has been no report of spinal invasion in cattle. ...
A 7-month-old Holstein heifer presented with posterior paresis and megaesophagus. At post mortem examination, a nodular tumor was found attached to the thoracic wall and the eighth to eleventh thoracic vertebrae, adjacent to the left posterior pulmonary lobe. The tumor was diagnosed as rhabdomyosarcoma by histology. This is a very rare case of spinal infiltration of rhabdomyosarcoma in cattle.
Background: Lumbosacral spinal lipomas and lipomyeloceles are usually identified in early childhood. Terminology, histopathology, and diagnosis for these malformations can be confusing. Materials and Methods: This is a PubMed review with comparison of embryology, gross, and histopathology, and reporting requisites for these and related closed spinal malformations. Results: The spinal lipoma group (congenital spinal lipomatous malformations) includes subcutaneous, transdural, intradural, and noncontiguous malformations stretching through the entire lower spinal region. This lipomyelocele trajectory overlaps the embryonic tail’s caudal eminence. Histopathologically, the lipomyelocele spectrum is a heterogeneous, stereotypical set of findings encountered from dermis to spinal cord. Diagnosis requires detailed correlation of images, intraoperative inspection, and histopathology. Conclusions: Appropriate terminology and clinicopathologic correlation to arrive at a diagnosis is a critical activity shared by pathologist and clinician. Prognostic and management differences depend on specific diagnoses. Familial and genetic influences play little if any role in patient management in closed spinal malformations.
Primary intraspinal sarcomas are very rare in children. Rhabdomyosarcoma (RMS) is a highly aggressive and rapidly growing sarcoma with skeletal origin that occasionally appears in the spinal epidural space.
We report a 13-year-old girl who presented with back pain, progressive paraparesis, and urinary retention. She had muscular weakness in her lower extremities and absent deep tendon reflex. An epidural dumbbell-shape mass at T11-T12 level was observed on MRI. The patient underwent T12 hemilaminectomy, partial T11 hemilaminectomy, and right facetectomy. A large, firm, dark-red vascular epidural tumor was found compressing the cord. The tumor which extended to intracanal and foraminal parts was removed completely.
Histopathological examination revealed undifferentiated small round and oval tumoral cells. Immunohistochemical staining was positive for vimentin, desmin, and myogenin. The final diagnosis was alveolar RMS. She received radiotherapy and chemotherapy by vincristine, actinomycin-D, doxorubicin, and ifosfamide. The patient was in good condition with no tumor recurrence or metastasis at the 1-year follow-up.
Sarcomas that arise from within the spinal canal are rare, particularly within the pediatric population. In general, these primary intraspinal sarcomas are highly aggressive, posing unique treatment challenges with respect to surgery and choice of adjuvant therapy. The goal must be to obtain the most complete resection possible to minimize the risk of recurrence and metastasis, while preventing potential neurological deficits that may result from aggressive surgery.
Among these primary intraspinal sarcomas are malignant peripheral nerve sheath tumors and members of the Ewing sarcoma family of tumors. The authors present 3 cases of unique spinal sarcomas in children—2 malignant peripheral nerve sheath tumors in patients without neurofibromatosis and an intradural extraosseous Ewing sarcoma arising from the sensory component of a lumbar spinal nerve—and discuss their management and outcome with a review of the current literature.
