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Open access

Spinal epidural lipomatosis in a pediatric patient with a malignant brain tumor: illustrative case

Reed Berlet, Daphne Li, and John Ruge

BACKGROUND

Spinal epidural lipomatosis (SEL) in pediatric patients with concomitant malignant brain neoplasms is rare and can present with rapid deterioration in neurological function.

OBSERVATIONS

A 4-year-old boy with SEL became paraplegic 4 months after completion of chemoradiation for his previously resected, intracranial atypical teratoid rhabdoid tumor. The patient presented with rapid deterioration in lower extremity sensory and motor function, which, given his oncological history, was concerning for disease progression. Of note, 8 months prior, the patient was started on corticosteroid therapy for respiratory dysfunction. Magnetic resonance imaging revealed significant progression of lumbosacral SEL requiring surgical decompression with subsequent neurological improvement.

LESSONS

When evaluating pediatric patients with primary or metastatic brain tumors with new or worsening myelopathy and motor or sensory deficits, it is important to consider SEL.

Open access

Pineal parenchymal tumor of intermediate differentiation with late spinal dissemination 13 years after initial surgery: illustrative case

Hiroyuki Kato, Takafumi Tanei, Yusuke Nishimura, Yoshitaka Nagashima, Motonori Ishii, Tomoya Nishii, Nobuhisa Fukaya, Takashi Abe, and Ryuta Saito

BACKGROUND

Pineal parenchymal tumors of intermediate differentiation (PPTIDs) are rare in the pineal gland. A case of PPTID that disseminated to the lumbosacral spine 13 years after the total resection of a primary intracranial tumor has been reported.

OBSERVATIONS

A 14-year-old female presented with headache and diplopia. Magnetic resonance imaging revealed a pineal tumor that induced obstructive hydrocephalus. A biopsy and endoscopic third ventriculostomy were performed. Histological diagnosis revealed a grade II PPTID. Two months later, the tumor was removed via craniotomy because the postoperative Gamma Knife surgery was ineffective. Histological diagnosis confirmed PPTID, although the grade was revised from II to III. Postoperative adjuvant therapy was not performed, because the lesion had been irradiated and gross total tumor removal was achieved. She has had no recurrence in 13 years. However, pain around the anus newly appeared. Magnetic resonance imaging of the spine revealed a solid lesion in the lumbosacral spine. The lesion was subtotally resected, and histological diagnosis revealed grade III PPTID. Postoperative radiotherapy was performed, and she had no recurrence 1 year after radiotherapy.

LESSONS

Remote dissemination of PPTID can occur several years after the initial resection. Regular follow-up imaging, including the spinal region, should be encouraged.

Open access

Surgical outcome of a patient with Bertolotti’s syndrome in whom the established Castellvi classification system failed: illustrative case

Richard J. Chung, Camryn Harvie, John O’Donnell, Sarah Jenkins, and Arthur L. Jenkins III

BACKGROUND

Bertolotti’s syndrome is a condition of the lower back and/or L5 distribution leg pain caused by a lumbosacral transitional vertebra (LSTV). Diagnosing the LSTV as the cause of the symptoms and condition is essential for accurate management of this syndrome. Castellvi’s classification system is widely accepted for LSTV anatomy, but it measures only one aspect of transitional anatomy and was intended primarily to identify target-level disk herniations.

OBSERVATIONS

In this case, the Castellvi classification system failed to identify the patient (with 2 years of back and L5 pain) as having an LSTV, even though he displayed LSTV-like anatomy because both L5 transverse process heights measured less than 19 mm. He attained brief but significant relief from bilateral injections into the L5–S1 transverse/ala region and underwent a minimally invasive bilateral decompression of L5–S1 with almost complete relief of his symptoms maintained more than 6 months postoperatively.

LESSONS

Given that the patient gained significant relief from treatment of transitional anatomy that failed to be identified using Castellvi’s classification system, this case suggests that transverse process height may not be adequate or even the most clinically relevant indicator in identifying LSTV anatomy, which is a precursor to the diagnosis of Bertolotti’s syndrome.

Open access

Presacral mature cystic teratoma associated with Currarino syndrome in an adolescent with androgen insensitivity: illustrative case

Grant Koskay, Patrick Opperman, Frank M. Mezzacappa, Joseph Menousek, Megan K. Fuller, Linden Fornoff, and Daniel Surdell

BACKGROUND

Currarino syndrome is a rare disorder that classically presents with the triad of presacral mass, anorectal malformation, and spinal dysraphism. The presacral mass is typically benign, although malignant transformation is possible. Surgical treatment of the mass and exploration and repair of associated dysraphism are indicated for diagnosis and symptom relief. There are no previous reports of Currarino syndrome in an androgen-insensitive patient.

OBSERVATIONS

A 17-year-old female patient presented with lack of menarche. Physical examination and laboratory investigation identified complete androgen insensitivity. Imaging analysis revealed a presacral mass lesion, and the patient was taken to surgery for resection of the mass and spinal cord untethering. Intraoperative ultrasound revealed a fibrous stalk connecting the thecal sac to the presacral mass, which was disconnected without the need for intrathecal exploration. The presacral mass was then resected, and pathological analysis revealed a mature cystic teratoma. Postoperatively, the patient recovered without neurological or gastrointestinal sequelae.

LESSONS

Diagnosis of incomplete Currarino syndrome may be difficult but can be identified via work-up of other disorders, such as androgen insensitivity. Intraoperative ultrasound is useful for surgical decision making and may obviate the need for intrathecal exploration during repair of dysraphism in the setting of Currarino syndrome.

Open access

Giant sacrococcygeal teratoma in a neonate: illustrative case

Durga Neupane, Alok Dahal, Nimesh Lageju, Lokesh Shekher Jaiswal, Nimesh Bista, and Aakriti Sapkota

BACKGROUND

Sacrococcygeal teratomas (SCTs) are tumors that emerge in the sacrococcygeal area and contain tissue from all three germ layers. SCT affects about 1 in every 35,000–40,000 live births, with malignant transformation becoming more common as the patient gets older. Ultrasound helps in prenatal diagnosis. Surgical resection is the mainstay of treatment.

OBSERVATIONS

A couple gave birth to a neonate with a small mass over his sacral region that progressively increased in size. Diagnostic magnetic resonance imaging was performed, and a diagnosis of giant SCT was established. Complete resection with flap reconstruction was performed. In regular follow-up, he is in a good state of health.

LESSONS

One of the most common tumors in infancy, SCT should be carefully diagnosed. SCT is often confused with neural tube defects such as myelocystocele or myelomeningocele. Complete resection with appropriate reconstruction can ensure better treatment, and close follow-up until adulthood is recommended to keep a close view on its possible recurrence and to improve prognosis. Postoperative complications such as infection, bleeding, and urethral complications should be carefully watched.