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... 1: A 15-year-old boy presented with urine & fe- cal incontinence since birth. MRI showed Bifid L5, sacro-coccygeal dysplasia & a tethered cord at L5 with a fatty mass compressing filum terminale which was ex- cised (Figure 1). Grossly the mass was a 5 cm soft tissue. On cut section fibro fatty tissue with fibrous streaks seen. Microscopically it was a non capsulate hamartoma, of fat, skeletal muscle, collagen and with a fragment of urothe- lium (Figure 2). Case 2: A 10-day boy presented with a 2 cm discol- ouration of skin suggestive of a nevus and a dermal sinus at S1 area with no bony deficit (Figure 3). Surgery showed a subcutaneous mass of 3 cms soft tissue with a tethered cord at S1 area, which on microscopy showed a fibrofatty hamartoma of collagen & fat (Figure 4). Case 3: A 10-month boy had a soft swelling at lum- bosacral area since birth. MRI showed a spinal dys- raphism at L4. A 7 cm skin covered mass with a central small bony structure of 0.6 cm and a tethered cord was excised ( Figure 5). On cut section fibro fatty tissue with fibrous streaks seen. Histology showed a benign osteoli- poma ( Figure 6). Case 4: A 4-month-old girl with right Club foot and progressive weakness of legs since last 2 months. MRI showed spina bifida at L5 and a tethered cord with an attached 9 cms capsulate mass (Figure 7). The mass was excised and histology showed a lipoma with a few nerve fibers attached to its surface (Figure 8). Case 5: A 7-year-old boy, with urine & fecal inconti- nence since birth had a swelling of 3 cms at sacral area since birth. MRI showed a bony deficit measuring 0.25 cm noted in S2 with a tethered cord beneath the mass. A polypoid mass of 3 cms composed of collagen extending to deep surgical margin was received (Figure ...
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... Clinical presentation depends upon the location and extent of spinal cord and / or, nerve root compression [ 1,[3][4][5][6][7][8][9][10] . ...
... MRI is the diagnostic test of choice to evaluate the relationship of the lesion with surrounding neurovascular structures and for operative planning [1,7,11] . Early detection and timely intervention results in favourable outcome [5,6,[8][9][10] . ...
... Their cutaneous and ocular stigmata helps in early evaluation of this rare entity [7,11,12] . However, association with Klippel feil syndrome, spinal giant cell xxx angioblastomas, Proteus syndrome, pseudopancreatic cyst have also been reported [5,8,13] . During review of the previously reported literature, only three documented cases were found having no features of such association [1,9,10] . ...
Background: Spinal hamartoma is an extremely rare lesion for symptomatic spinal cord compression in adult population.
Without any association of spinal dysraphism and neurofibromatosis, only three cases have been reported in the
literature.
Case description: A 40-year-old man presented with lumbago femorica for 6 months. Gradually the symptoms worsened
and he developed features of Cauda equina syndrome. Magnetic Resonence Imagimg demonstrated a
heterogeneously hyperintense intradural extramedullary mass in both T1 and T2WI, involving conus medullaris
and cauda equina, opposite to L1. Patient underwent near total resection of lesion through posterior midline
approach. Histopathological features were consistent with hamartoma. Patient neurologically improved and follow
up MRI at 6 months showed static small nodular tumor residue.
Conclusion: To the best of our knowledge, current report is the fourth case of spinal hamartoma in adult male, without any
association of spinal dysraphism and neurofibromatosis .This may be the first reported case from Bangladesh.
Keywords: Hamartoma, Neurofibromatosis, Spinal; Dysraphism
... Congenital midline spinal hamartoma is a rare spine tumor-like proliferation that mostly occurs during childhood. [1][2][3] Hamartoma consists of mature, well-differentiated ectodermal and mesodermal elements that present in an abnormal location. 1,4,5 Although the prevalence of midline spinal hamartoma is not exactly known, this tumor-like proliferation is less common than the other congenital tumor lesions involving the spine, such as lipomas, teratomas, dermoid cysts, and epidermoid cysts. ...
... [1][2][3] Hamartoma consists of mature, well-differentiated ectodermal and mesodermal elements that present in an abnormal location. 1,4,5 Although the prevalence of midline spinal hamartoma is not exactly known, this tumor-like proliferation is less common than the other congenital tumor lesions involving the spine, such as lipomas, teratomas, dermoid cysts, and epidermoid cysts. 1,[4][5][6] This lesion is usually associated with other conditions, such as neurofibromatosis type 1, spinal dysraphism, or without involving any of the associated conditions. ...
... 1,4,5 Although the prevalence of midline spinal hamartoma is not exactly known, this tumor-like proliferation is less common than the other congenital tumor lesions involving the spine, such as lipomas, teratomas, dermoid cysts, and epidermoid cysts. 1,[4][5][6] This lesion is usually associated with other conditions, such as neurofibromatosis type 1, spinal dysraphism, or without involving any of the associated conditions. [1][2][3][7][8][9] Midline spinal hamartomas that associated with spinal dysraphism commonly presenting with the normal overlying skin, skin dimple, hyperpigmented skin, subcutaneous mass, or cutaneous angioma. ...
Background:
Congenital midline spinal hamartoma is a very rare tumor-like proliferation that mostly occurred during childhood. It consists of mature, well-differentiated ectodermal and mesodermal elements that present in an abnormal location and mostly associated with neurofibromatosis type 1 and spinal dysraphism.
Case description:
A 5-month-old male patient presented with a complaint of a bump over the lower back. The bump is already presented since birth with the size is approximately 3 cm in diameter and growth slowly. There are no other associated symptoms besides the bump on the lower back. We found a subcutaneous mass with associated skin dimple located on the midline of the lower back. CT scan of the spine shows a mass that arose from inside the dura and a closed spinal dysraphism from L3 vertebra to the sacrum. A subtotal resection was performed and the histopathology feature showing fat cells, cartilage, skeletal muscle fibers, nerve, and blood vessel. After 6 months follow up, no symptoms nor neurological deficit was present, and no further growth is shown on the latest CT scan.
Conclusions:
Congenital midline spinal hamartoma is very rare and seldom addressed. Mostly patient presenting with a cosmetic defect and rarely with associated symptoms. Tissue biopsy is still the best modality to definitively diagnose hamartoma and to exclude other diagnoses. Surgical excision of the tumor is still the mainstay treatment especially for patients who are not neurological intact and to correct the cosmetic skin defect.
... Castillo et al. argued that both ways could be effective in the process [1]. In the study of Rao et al., it is stated that the mechanism of formation may be due to incomplete fusion in the neural tube [4]. It has also been described that the protrusion in the spinal cord may be caused by a traction of the dermal sinus tract [1]. ...
Two horses euthanized for neurologic deficits were diagnosed with hamartomatous myelodysplasia of the spinal cord. One was a 5-week-old Holsteiner colt exhibiting spasms of muscle rigidity in the extensor muscles of the limbs and epaxial muscles, and the other was a 3-year-old Thoroughbred colt exhibiting progressive ataxia and hypermetria in the pelvic limbs. Each had focal disorganization of the white and gray matter of the spinal cord forming a mass interspersed with neurons, glial cells, and disoriented axon bundles. In the Holsteiner colt, the mass was at the level of C5 and included islands of meningeal tissue contiguous with the leptomeninges. The mass occluded the central canal forming hydromyelia cranial to the occlusion. In the Thoroughbred colt, the mass was at the level of L1 on the dorsal periphery of the spinal cord and did not involve the central canal.
