Primary orbito-cranial adenoid cystic carcinoma with torcular metastasis: a case report and review of the literature

Primary orbito-cranial adenoid cystic carcinoma with torcular metastasis: a case report and review of the literature

JOCN-126.QXD 2/25/01 3:54 PM Page 173 Orbital adenoid cystic carcinoma 173 Primary orbito-cranial adenoid cystic carcinoma with torcular metastasi...

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Primary orbito-cranial adenoid cystic carcinoma with torcular metastasis: a case report and review of the literature J. Sousa MS, R. R. Sharma MS DNB, A. Delmendo MD, S. J. Pawar MCH Department of Neurosurgery, Khoula Hospital, Muscat, Sultanate Of Oman

Summary Adenoid cystic carcinoma is a rare neoplasm arising from the exocrine glands such as salivary glands, lachrymal glands, upper respiratory tract, breast or uterine cervix. Intracranial involvement is commonly from direct skull base involvement, although metastasis may rarely be seen. The predisposition of the adenoid cystic carcinoma for perineural and perivascular invasion is the prime reason for the locally invasive character of the tumour, often extending into the cranium via foramina at the skull base. The authors report a case of primary orbito-cranial extradural adenoid cystic carcinoma and cranial metastasis away from the primary site. This patient initially had a local excision, and later an exenteration of the right eye followed by with radiotherapy. Within months she presented with an extradural cranial recurrence, distant torcular metastasis without any neurological deficit. Craniotomy and radical excision was undertaken as these tumours have slow growth rates, and long term survival of these patients even in the presence of local recurrence and metastasis has been well documented. © 2001 Harcourt Publishers Ltd Journal of Clinical Neuroscience (2001) 8(2), 173–176 © 2001 Harcourt Publishers Ltd DOI: 10.1054/jocn.2000.0754, available online at on

Keywords: orbito-cranial tumour, adenoid cystic carcinoma, lachrymal gland, intracranial metastasis, metastasis, skull base surgery Received 2 February 2000 Accepted 22 March 2000 Correpondence to: Dr Rewati Raman Sharma, P.O. Box 397, Al–Harthy Complex, Qurum, 118, Muscat, Sultanate of Oman. Tel.: ;968 567339/ 562863; Fax: ;968 567339; E-mail: [email protected]

INTRODUCTION Adenoid cystic carcinoma (ACC) is a malignant tumour arising from the salivary glands, lachrymal glands, upper respiratory tract, breast or uterine cervix.1–6 ACC constitutes 1% of all head and neck malignancies.7 It commonly presents with delayed metastasis to the lungs, bone and the liver.1,3–6,8–11 The 5 year and 20 year survival rate for the ACC tumours located in the head and neck has been reported as 62–75% and 13% respectively.3,5,8,9,12 Prognosis depends on the histological grade of the tumour. The authors report a rare case of primary orbito-cranial adenoid cystic carcinoma and torcular metastasis with interesting clinicoradiological features, and a review of the literature. CASE REPORT A 34 year old lady presented with complaints of progressive diplopia, proptosis and deviation of the right eye for approximately © 2001 Harcourt Publishers Ltd

2 months’ duration in May 1998. She underwent a total excision of an extra-axial orbital mass via a lateral orbitotomy. The histological examination was reported as an adenoid cystic carcinoma arising from the lachrymal gland. She received a course of post-operative radiotherapy. However, 8 months later, she presented with a recurrence of the tumour with erosion of the orbital apex and the superior orbital fissure, which was detected on a follow up magnetic resonance imaging (MRI) scan. She therefore underwent an exenteration of the eye, with excision of the posterolateral orbital wall and the extradural tumour in the fronto-temporal region, which was followed by a second course of radiotherapy. One year later, she presented with a frontal bosselated lesion, which on investigation by MRI and computed tomography (CT) scans had revealed a large extradural temporofrontobasal recurrence with infiltration of the overlying bone, and compression of the right frontal lobe (Fig. 1). The scans also revealed distant metastasis in the region of the torcula (Fig. 2). X-ray chest, bone scans and abdominal ultrasound did not reveal any other site of metastasis. She was reoperated on and a wide frontotemporal craniotomy with excision of a large vascular extradural tumour along with the involved cranium was carried out. The dura was intact, but the bone flap revealed gross infiltration by the tumour. She is undergoing a further course of radiotherapy and is neurologically well preserved. Histological examination of the tumour showed a neoplasm with cells arranged in groups or occasionally in cribriform pattern suggestive of ACC with a predominantly solid pattern. Histological examination of the bone revealed extensive bone infiltration by the tumour.

DISCUSSION Although ACC commonly arises from the major salivary glands, usually the parotid gland, the tumour may also be found arising from the minor salivary glands, lachrymal gland, upper respiratory tract, breast, or the uterine cervix.1–6 ACC of the lachrymal gland invading the cranium and presenting as an extradural mass is rare. ACC is characterised by slow growing local recurrences with delayed metastasis to the lung, liver, and bones.1,3–6,8–11 Histologically, ACC is broadly classified into three categories: cribriform, tubular and solid;3,5,13–15 in addition to the tumour size, site and histological type, the histological grade is of important prognostic value which depends upon the solid component of the tumour.16,17 ACC has been graded into three grades depending upon the ratio of the solid component with the tumour: Grade I, no solid pattern, Grade II, less than 30% solid component and Grade III, with more than 30% solid component.7,15,18 It has been shown that patients with grade I have a 39% 15 year survival rate, while those with grade II tumours have a 26% survival rate, and those with grade III tumours have a 5% 15 year survival rate.15,19 ACC has a characteristic tendency to invade perivascular and perineural tissues.2,10,11,15 Tumour from the salivary glands, upper respiratory tract, paranasal sinuses commonly invade the skull base,20,21 with a reported incidence of 4–22%.1,11,22–26 The tumour has a tendency to involve the infratemporal and middle cranial fossae before extending towards the petrous apex, cavernous sinus, and orbital apex.10,27 Shotton et al.20 in cases of ACC of the nasopharynx, proposed that the cranial base invasion is along three routes, namely, eustachian tube, mandibular and maxillary nerves, and the internal carotid artery. Gromley et al.10 in the largest reported series of invasive ACC, reported 16 cases with cranial base and intracranial extension. However, isolated intracranial metastasis are very rare. Kazumoto et al.28 has reported that there are only six such confirmed cases in the world literature. The onset of symptoms is usually delayed, with the duration between the presentation and the onset of symptoms varying from Journal of Clinical Neuroscience (2001) 8(2)


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Fig. 1 MRI scans showing a hypo-intense right frontotemporal mass on T1-weighted image (A), which is iso-intense on T2-weighted image (B), and enhances on contrast (C).

Fig. 2 CT scan showing intradiploic cranial metastasis in the region of the torcula with bone erosion in the right fronto-temporal region.

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2.7 to 5 years.6,24 Initially, there is a paucity of symptoms, with the patients commonly complaining of epistaxis and a mass, however, with the skull base involvement the symptoms may dramatically change to eustachian tube dysfunction, trigeminal nerve involvement, and ocular motility dysfunction.20,23,24,29 In our case, the patient’s initial recurrence was diagnosed on a follow up MRI, while in the second instance she presented with a bosselated nontender frontal mass. Commonly used modalities of investigation in these patients include CT scan and MRI. CT scan with bone window settings allows for better definition of the bony involvement although it may not identify small tumours at the base of the skull without extensive bony changes even though they may have intracranial involvement.30 However, MRI with gadolinium enhancement clearly demonstrates the tumour with the associated perineural course.10 MRI also demonstrates the involvement of the cavernous sinus and the brain tumour interface.31,32 However, some authors22,33 have pointed out that there are no specific CT and/or MRI findings to differentiate meningiomas from intracranial adenoid cystic carcinoma. Both lesions on MRI scans are hypointense on T1, iso- to hyperintense on T2, and demonstrate a

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surgery at an early stage may offer a long term survival when combined with radio-chemotherapy. One such case is presented with the CT and MRI findings.




5. 6. Fig. 3 MRI scan showing the ‘dural tail sign’ in an intracranial extradural ACC.

7. 8.

bright homogenous enhancement on contrast administration,22,34 as also seen in our patient (Fig. 1). Even the ‘dural tail sign’ synonymous with meningiomas35 has been described in other dural tumours including adenoid cystic carcinoma22,36 as was noted in our case (Fig. 3). Angiography also has an important role, especially to identify the vasculature of the tumour. While radical surgery is the main line of treatment of these tumours,7,20,23–25 their infiltrative nature makes this very difficult. Shotton et al.20 have designed a staging system for ACC of the nasopharynx which assists in planning of the surgical approach for these tumours. In this classification, T1 tumours are confined to one primary nasopharyngeal site, T2 tumours have more than one nasopharyngeal site with bony invasion into the paranasal sinuses, T3 tumours have peritubal infiltration with infratemporal fossa invasion, and T4 tumours have intracranial extension. At surgery, a frontotemporal craniotomy with trans-zygomatic exposure provides good access to the intracranial tumour including cavernous sinus involvement. Addition of an orbitozygomatic osteotomy can give access to the intraorbital component of the tumour. The infratemporal fossa, peritubal space and the pterygopalatine fossa can be readily accessed through a subtemporal-infratemporal approach.37,38 Most recurrences occur within the first 3 years.24,39 Recent studies have shown that with radiotherapy, local control, but no cure, can be achieved in ACC.5–8,10,11,15,23–25,39–43 Tumour shrinkage has also been reported in patients with intracranial adenoid cystic carcinoma following radiotherapy.23 Chemotherapy has also been shown to improve survival in these patients.6,23,40,44,45 Although no firm data are available as regards to ACC of the lachrymal gland, intracranial involvement suggests an advanced stage. A mean survival of 49 months (range, 6–98 months) was reported by Gormley et al.10 most of whose patients had large intracranial mass lesion. This may suggest that the use of palliative surgery should be considered for such patients with large intracranial lesions, even if the extent of local disease precludes a surgical cure.10 CONCLUSION ACC of the lachyrmal gland is a rare orbital malignant neoplasm causing early involvement of the orbital contents and spreads contiguously to the cranium and via the perineural and perivascular routes to the intracranial contents. Radical skull base © 2001 Harcourt Publishers Ltd




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Extradural spinal cavernous haemangioma: case report and review of the literature Gordon A. O. Appiah1 MB CHB, Neville W. Knuckey1 MD FRACS, Peter D. Robbins2 MBBS FRACPA 1

Neurosurgical Department, Sir Charles Gairdner Hospital, Nedlands, WA, Western Australian Centre for Pathology and Medical Research, QEII Medical Centre, Nedlands, WA, Australia 2

Summary Cavernous haemangiomas (cavernomas) are uncommon vascular malformations of the central nervous system (CNS). They occur in both sporadic and familial forms and may involve any site in the CNS. Spinal cavernomas are less common than intracerebral lesions, and examples in the spinal epidural space are rare. A case of a solitary sporadic spinal extradural cavernoma in a

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41 year old male which presented as progressive lower limb numbness and weakness is reported. The literature regarding spinal cavernomas is reviewed and the symptomatology, diagnostic evaluation, pathology, management and prognosis of these lesions are discussed. © 2001 Harcourt Publishers Ltd Journal of Clinical Neuroscience (2001) 8(2), 176–179 © 2001 Harcourt Publishers Ltd doi: 10.1054/jocn.2000.0756, available online at on

Keywords: spinal cavernous haemangioma, extradural, intradural extramedullary, intramedullary Received 22 February 2000 Accepted 22 March 2000 Correspondence to: N. W. Knuckey, Neurosurgical Department, SCGH, Nedlands, WA 6009, Australia. Tel.: ;61 8 93462865; Fax: ;61 8 93463824; E-mail: [email protected]

INTRODUCTION Cavernous haemangiomas (cavernomas) are uncommon vascular malformations of the central nervous system (CNS). They most commonly occur in the cerebral hemispheres but may involve any part of the neuraxis including the spine.1,2 In the spine, the majority of vascular lesions are secondary extensions of vertebral haemangiomas.3 Extradural haemangiomas represent 4% of all spinal epidural tumours and 12% of all intraspinal haemangiomas.4 Intradural cavernomas of the spine are rare lesions.2 A case of spinal epidural cavernous haemangioma is presented and the English literature regarding spinal cavernomas reviewed. The symptomatology, pathology, neuroradiology, management and prognosis as highlighted by the 157 reported cases1–3,5–37 are described and summarised. The various forms of isolated spinal cavernous haemangiomas (intramedullary, intradural extramedullary and extradural) are discussed. The need to include cavernomas in the differential diagnosis of various spinal conditions is emphasised.

CASE REPORT A 41 year old male was referred to the Sir Charles Gairdner Hospital, Perth, Australia in June 1999. He presented with a 12 month history of back pain which radiated to the anterior chest and bilateral leg numbness. He also noticed weakness in his lower limbs for 2 months which became progressively worse in the 2 weeks prior to presentation. There was no bladder or bowel dysfunction and there was no past history of spinal trauma, infection or tumour. On examination, he was hypertonic and hyperreflexic in his lower limbs with an extensor right plantar response. There was reduced sensation to light touch in the lower chest. Posterior column function was normal. Magnetic resonance imaging (MRI) of his thoracic spine demonstrated a posterior extradural mass at T8-9 level with compression of the cord. The mass extended into the intervertebral foramen. T1-weighted images are illustrated in Figs 1–3. T2-weighted images revealed increased signal intensity. MRI of the brain was normal. T8-9 laminectomy and complete extirpation of a dark reddish lobulated lesion was performed in July 1999. Microscopic examination of the lesion revealed a collection of dilated, fibrous walled vascular spaces lined by unremarkable endothelium within epidural adipose tissue (Figs 4 and 5). Scattered central areas of sclerosis were associated with microscopic deposits of haemosiderin. He was asymptomatic on follow up 6 months later. © 2001 Harcourt Publishers Ltd