Craniospinal Dissemination of Clival Chondroid Chordoma

� Case Report www.jpgmonline.com Craniospinal Dissemination of Clival Chondroid Chordoma

Shinde SV, Monipanda K

Department of ABSTRACT Pathology, B. Y. L Nair Chondroid chordoma commonly presents as clival osseous and extradural mass. A 15-year-old boy presented Hospital, Mumbai, India with progressive visual deficit, headaches and diplopia since three years. Computed tomography (CT scan) Correspondence: showed a skull base tumour, but was wrongly reported at the time as chronic sphenoidal sinusitis and nasal Shinde Sweety polyps. In the past three months, he developed dysphagia, urinary retention and constipation. Terminally, he E-mail: [email protected] had weakness of all limbs. Fundoscopy showed optic atrophy. Temporal and spatial variation in symptoms led to a clinical diagnosis of multiple sclerosis with optic neuritis. Partial brain autopsy revealed small gelatinous tumour nodules in the subarachnoid space of middle cranial fossa encasing base of brain like arachnoiditis. Tumour deposits extended down into the spinal cord along the subarachnoid space as far as vision allowed. Received : 11-01-05 Histopathology and immunohistochemistry confirmed a diagnosis of chondroid chordoma. Awareness of this Review completed : 08-02-05 rare mode of dissemination will avoid misdiagnosis and delay in treatment. Accepted : 08-04-05 PubMed ID : 16333198 J Postgrad Med 2005;51:220-2 KEY WORDS: Craniospinal-chordoma-subarachnoid

hondroid chordoma commonly presents as clival tu­ cal diagnosis was Devic‘s disease (Multiple Sclerosis with op­ C mour in young individuals and has a favorable prog­ tic neuritis) with urethral stricture. The patient expired be­ nosis.[1] Drop metastases and dural dissemination usually fol­ fore Magnetic Resonance Imaging (MRI) could confirm the low multiple surgical interventions due to implantation or diagnosis. hematogenous spread.[2,3,4] The present case in a young boy showed a craniospinal dissemination of clival chondroid chor­ Partial brain autopsy revealed diffuse gelatinous tumour nod­ doma along the subarachnoid space in the absence of any sur­ ules encasing the base of the brain in a flat sheet, like arach­ gical intervention. noiditis [Figure 1] and causing local destruction of the optic nerve and pituitary. Tumour deposits extended down the Case History foramen of Magnum along the subarachnoid space as far as vision allowed. A 15-year-old boy presented three years ago with diplopia and bilateral lateral rectus palsy following a minor nasal trauma. Histopathology showed a lobular tumour [Figure 2] with chon­ Computed tomography (CT) scan at the time was reported as droid and chordoid areas. Chordoid foci showed typical being unremarkable. In the following six months, the patient physalipherous and cuboidal cells in cords and trabeculae. The started having intermittent throbbing frontal headaches with cells were Periodic Acid Schiff (PAS) positive and diastase di­ progressive visual deficit. The CT scan showed a skull base gestible. These areas were merging with chondroid matrix with tumour, but was reported at that time as nasal polyps and atypical cells in lacunae [Figure 3], thus satisfying Heffelfinger’s chronic sphenoidal sinusitis. During the previous three months, critera.[1] The matrix was Alcian blue positive. Psammoma he had dysphagia and suffered from an inability to initiate uri­ bodies and metaplastic bone formation were seen. Immuno­ nation and defecate. Terminally, he came to Mumbai with uri­ histochemistry found the tumour to be strongly positive for nary retention, weakness of all limbs and projectile vomiting. EMA (epithelial membrane antigen), S100, vimentin and Neurological examination revealed hypotonia of all limbs along cytokeratin, while GFAP (glial fibrillary acid protein) positivity with areflexia and sensory loss below thoracic(T)6 level. Cer­ was focal and weak, thus confirming the diagnosis of a chon­ ebrospinal fluid (CSF) examination was unremarkable. Fun­ droid chordoma. doscopy revealed bilateral optic atrophy and macular oedema. Complete haemogram revealed only mild anaemia (Haemo­ Discussion globin 8.3 gm%). Ultrasonography(USG) of the abdomen re­ vealed bilateral hydroureter, mild hydronephrosis and cystitis. Chordomas are tumours arising from rests of primitive noto­ Urine examination showed trace proteins and sugar and few chord along the craniospinal axis, especially at the cranial and pus cells. Renal and liver function tests were normal. USG sacral end.[1] Chondroid chordoma, first described by abdomen and x-ray chest did not show any focal lesion. Clini­ Heffelfinger in 1973, is a variant of chordoma that straddles

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the bridge between conventional chordoma and chondrosar­ coma.[1] Twenty-eight to thirty-four per cent of skull base chor­ domas are of the chondroid variety.[1]

Common clinical presentations[1] of clival chordoma include diplopia, visual defect, headache, VI-XII cranial nerve palsies, hypopituitarism and intranasal mass.[5] Rarely, does it present as pure intradural chordoma.[6] Drop metastases and subarach­ noid dissemination from clival origin to cervical and thoraco­ lumbosacral regions usually occurs late in the course of the disease and after multiple surgical and radiation therapies.[2,3,4] This route of spread is either due to surgical implantation or hematogenous dissemination[2,3,4] and is more common with anaplastic chordomas.[3]

On hindsight, our case had all the typical features of a clival Figure 1: Chondroid Chordoma-Gross photograph showing subarachnoid space-occupying lesion, though they were initiated by minor nodular deposits of gelatinous tumor (arrow) encasing the base of the brain trauma. Dysphagia and nasal polyps were probably due to na­ in a sheet-like manner resembling arachnoiditis sopharyngeal spread of clival tumour. Weakness, hypotonia, areflexia could be attributed to cervico-thoraco-lumbar spread, while bladder and bowel symptoms were due to the sacral spread of the tumour. The cranial symptoms preceded quadriparesis, which preceded urinary symptoms. This se­ quence suggests primary craniospinal subarachnoid progres­ sion of tumour, since our case had no surgical intervention. Surprisingly, the CSF examination did not detect any malig­ nant cells. The patient had no symptoms of hypopituitarism and no metastases were detected by USG or on chest x-ray.

On CT scan, chordoma presents as a destructive bone lesion with periosteal elevation and a solid-cystic soft tissue mass with foci of calcification.[1,6,7] The chondroid variety calcifies more frequently than the conventional type.[1] On MRI, it is hyperintense and enhances heterogeneously with contrast material.[7] Myelography is preferred to MRI for diagnosis of Figure 2: Chondroid Chordoma- Distinctive lobular appearance (arrow) of subarachnoid nodules, since there may be loss of contrast be­ the tumor on light microscopy. 50 x, Hematoxylin-Eosin stain (HE) tween lesions and CSF on MRI.[4] In our case, the tumour did not form a tumour mass, but encased the base of brain like a flat sheet resembling arachnoiditis. Perhaps, this is why it was not picked up on CT scan.

Differential diagnoses of skull base chondroid chordoma in­ clude myxoid chondrosarcoma, chordoid meningioma, metastases of signet ring adenocarcinoma and chordoid glioma. Light microscopic features and immunohistochemistry can differentiate between these entities.[1,8-10]. Chondroid chordoma presents commonly in young females, has a lower recurrence rate and better survival as compared to conventional chor­ doma.[1] This prognosis is attributed to chondroid differentia­ tion in notochord.[1] Our case however showed a relatively rapid decline.

The present case highlights a rare presentation of chondroid chordoma as flat sheet-like encasing of base of brain resem­ Figure 3: Craniospinal Dissemination of Clival Chondroid Chordoma bling arachnoiditis. The tumour disseminated through the

J Postgrad Med September 2005 Vol 51 Issue 3 221 � � Shinde et al: Clival chondroid chordoma subarachnoid space along the craniospinal axis in the absence Neuroradiology 1999;41:504-7. 3. Hall WA, Clark HB. Sacrococcygeal chordoma metastatic to the brain with of any interventional procedures that could have initiated review of the literature. J Neuro Oncol 1995;25:155-9. metastases. Compression and infiltration of the spinal cord 4. Krol G, Sze G, Arbit E, Marcove R, Sundaresan N. Intradural metastases of chordoma. AJNR 1989;10:193-5. with multiplicity of symptoms in time and space made it mimic 5. Scartozzi R, Couch M, Sciubba J. Chondroid chordoma of the nasal septum. a demyelinating disorder. Clinical awareness of this mode of Arch Otolarygol Head Neck Surg 2003;129:244-6. 6. Steenberghs J, Kiekens C, Menten J, Monstrey J. Intradural chordoma with­ presentation will prevent delay in diagnosis and treatment. out bone involvement. J Neurosurg Spine 2002;97:94-7. 7. Meyers SP, Hirsch WL, Curtin HD, Barnes L, Sekhar LN, Sen C. Chordomas of the skull base: MR features. Am J Neuroradiol 1992;13:1627-36. References 8. Rosenberg AE, Brown GA, Bhan AK, Lee JM. Chondroid Chordoma-A Vari­ ant of Chordoma-Morphologic and Immunohistochemical study. Am J Clin Pathol 1994;101:36-41. 1. Heffelfinger MJ, Dahlin DC, MacCarty CS, Beabout JW. Chordomas and 9. Couce ME, Aker FV, Scheithauer BW. Chordoid Meningioma A clinicopatho­ cartilaginous tumors at skull base. Cancer 1973;32:410-20. logic study of 42 cases. Am J Surg Pathol 2000;24:899-905. 2. Uggowitzer MM, Kugler C, Groell R, Lindbichler F, Redner H, Sutter B, et al. 10. Pasquier B, Peoc‘h M, Morrison AL, Gay E, Pasquier D, Grand S, et al. Chordoid Drop metastases in a patient with a chondroid chordoma of the clivus. Glioma of the third ventricle. Am J Surg Pathol 2002;26:1330-42.

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