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Show Abstract: An 83- year- old woman presented with right Departments of Ophthalmology ( SCL, AGL), Neurology ( AGL), , . . , . . . ,. , , ,. , . , Neurosurgery ( AGL), Otolaryngology ( SMG), and Pathology ( PAK), trigeminal paresthesias, Upper lid ptosis, and diplopia and University of Iowa Hospitals and Clinics, Iowa City, Iowa. Was found to have a right third Cranial nerve palsy. MRI Presented in part at the 2005 Frank B. Walsh Society Meeting, disclosed a light sphenocavernOUS mass with speckled high February 13, 2005, Copper Mountain, Colorado. signal on pre- contrast Tl and correspondingly low signal Supported in part by an unrestricted grant from Research to Prevent Qn J2 E n d o s c o p i c sphenoidotomy revealed malignant mela- Blindness, Inc., New York, New York. _ . , ,. . , ., , Address correspondence to Andrew G. Lee, MD, Department of n0ma- The P a t e l t underwent radiation therapy With complete Ophthalmology, 200 Hawkins Drive PFP, The University of Iowa Hospitals and protracted resolution of the palsy An extensive evalua-and Clinics, Iowa City, IA 52242; E- mail: andrew- lee@ uiowa. edu tion for a primary Source Was negative. The combination of high Tl signal and low T2 signal in a mass should suggest melanin. An exceedingly rare condition, this is the first English report of its presentation with a third cranial nerve palsy. ( J Neuro- Ophthalmol 2005; 25: 289- 292) An 83- year- old woman presented with new right upper lid ptosis, diplopia, and a warm sensation in the second trigeminal distribution on the right. She said she did not have a headache. Ocular and medical history was non-contributory. There was no previous personal or family history of cutaneous or systemic malignancy. She was taking one aspirin per day. She was widowed and did not smoke nor drink alcohol. Visual acuity was 20/ 30 OD and 20/ 20 OS. There was almost complete ptosis OD. The pupils measured 4 mm OU in darkness and 2 mm OU in light. There was no relative afferent pupillary defect. There was complete lack of supraduction, adduction, and infraduction but intact abduction and intorsion OD. The ductions were intact OS. There was a 60 prism- diopter exotropia in primary position. She was hyperesthetic to pinprick in the second trigeminal distribution on the right side of the face and reported a paresthesia " like a warm sensation" in this area. Slit lamp examination showed a cataract OD consistent with 20/ 30 acuity. The remainder of the ophthalmic examination, including intraocular pressure measurements and ophthalmoscopy, was normal OU. There was no temporal artery tenderness. The neurologic examination was normal. An erythrocyte sedimentation rate and C- reactive protein were normal. Computed tomography ( CT) of the sinuses showed opacification and expansion of the right sphenoid sinus and partial erosion of the posterolateral wall adjacent to the right cavernous sinus. Brain MRI revealed a lesion in the right cavernous and sphenoid sinuses that showed areas of hyperintensity on T1 corresponding to areas of hypointensity on T2 and mild homogenous contrast enhancement ( Fig. 1). CT of the chest, abdomen, and pelvis was unremarkable. Complete physical examination was normal. The differential diagnosis included cavernous sinus meningioma, lymphoma, metastatic cancer, and sphenoid sinus adenocarcinoma. A right endoscopic sphenoidotomy revealed that the sphenoid sinus lesion was covered with a layer of normal mucosa. A subtotal resection and biopsy were performed. The nidus of the mass appeared to be within the wall of the lateral sphenoid sinus, with extension into the adjacent cavernous sinus. The pathology showed an infiltrate of large polygonal cells with abundant cytoplasm. The nuclei were large and vesicular with prominent nucleoli. Irnmunohistochemistry for melanoma markers S- 100, HMB 45, and MART 1 was positive ( Fig. 2). Examination by a dermatologist was negative for cutaneous melanoma. Positron emission tomography ( PET) scan showed nonspecific uptake in the right upper abdominal quadrant, but no primary melanoma was identified. This PET scan finding was not believed to be consistent with metastatic melanoma, although it cannot be completely excluded. The patient was treated with 60 Gy of radiation ( cutaneous and mucosal) originate in the head and neck, but therapy in 30 fractions to the tumor bed with 54 Gy to the only 1% arise from the sinonasal tract ( 1,2). The nasal cavity sphenoid sinus. Within a few months of treatment, her is most frequently affected, followed by the maxillary sinus, ptosis had resolved. A repeat MRI three months after ethmoid sinus, frontal sinus, and sphenoid sinus ( 1). The treatment showed marked reduction in the size of the right melanoma is derived from melanocytes that normally cavernous sinus and sphenoid sinus lesion ( Fig. 3). She migrate as neural crest derivatives in ectodermally- derived denied diplopia despite a residual 12 prism- diopter intermit- mucosa ( 2,3). In a review of 377 cases of head and neck tent exotropia. She had significant improvement of her melanomas ( 2), the peak incidence was in the fifth to eighth trigeminal paresthesias with resolution of the warm feeling decade. The earliest symptoms were epistaxis and nasal she had described previously. As of her latest visit, eleven stuffiness ( 2,4,5). There have been only five reported cases months after treatment, she was asymptomatic. MRI showed of primary sphenoid sinus melanoma ( 4- 8). Of these, four minimal residual tumor with postoperative changes in the cases initially presented with neuro- ophthalmic complaints, sphenoid sinus, and there was no evidence of metastatic including diplopia, ptosis, visual field defects, or decreased disease. The PET scan was not repeated. The patient was visual acuity ( 4,6- 8). Each of these patients had spread of offered but refused colonoscopy. An oncologist examined the melanoma into the adjacent cavernous sinus ( Table 1). the patient and decided that, in the absence of any sug- The appearance of melanoma on MRI depends on the gestive symptoms, repeated evaluation was not required. amount of melanin in the tumor. Melanotic melanoma often The melanoma in our patient appears to have been shows a hyperintense signal on Tl and a hypointense signal primary to the sphenoid sinus. Primary malignant melanoma on T2. Amelanotic melanoma is hypointense or isointense of the paranasal sinuses is rare. Almost 20% of melanomas on Tl and hyperintense or isointense on T2. The hyperintense signal intensity seen on Tl- weighted 2. Manolidis S, Donald PI Malignant mucosal melanoma of the head images and hypointense signal on T2- weighted images is TwiltirS- Z ^ ^ ' ^ ^ *** " ^ ° f " Pati6ntS' ^ " ^ caused by melanin's paramagnetic properties ( 9,10). The 3. Zak FG, Lawson W. The presence ofmelanocytes in the nasal cavity. differential diagnosis for an increased Tl signal on MRI Ann Otol RMnolLaryngol 1974; 83: 515- 9. also includes fat, gadolinium, methemoglobin, proteinaceous 4- B u s i* a **?• P r i m a ^ m e l a n o m a o f t h e sPh e n o i d simis- Otolaryngol . . . '& . ' & F . Head Neck Surg 2000; 123: 748- 9. fluid, ferritin, copper, immature Calcium, magnesium, and 5_ C a r t e r T R pathologic quiz case 1. Malignant melanoma. Arch manganese complexes. Otolaryngol Head Neck Surg 1986; 112: 450- 3. Surgical resection is the treatment of choice for 6- PmoRiveroXKeituqwaYanezT. Melanoma of sphenoid sinus: case . presentation and review of literature. Acta Otorrinolaringol Esp melanoma ( 11). However, radiation therapy and chemo- 2004- 55- 45- 8 therapy, as primary or adjunctive treatments, have also been 1. shinbori T, Uyama E, Eto K, et al. An autopsy case of malignant used With disappointing results ( 2,11). Immunotherapy melanoma possibly originating in the sphenoid sinus. Rinsho . . . _ , , 11 • - 1 , Shinkeigaku I988; 28: 636^ l2. With interferon alpha or allogenic vaccine has not yet been 8 A s a n o K> S o b a t a E> Yamazaki K, et al. Malignant melanoma arising sufficiently evaluated ( 12). Local relapse OCCUrs in more from the sphenoidal sinus: case report. Neurol Med Chir ( Tokyo) than 50% despite treatment. Distant metastasis can occur 2000; 40: 329- 34. 1 nrirp • • • .1 1- .• 1 1-. 9. Warakaulle DR, Anslow R Differential diagnosis of intracranial and PET scanning is emerging as the diagnostic modality leslons w l t h M g h s l g n a l o n T 1 o r l o w s l g n a l o n T 2 . w e l g h t e d M R I for metastatic evaluation. In comparison to patients with ciin Radiol 2003; 58: 922- 33. CUtaneOUS melanoma, the prognosis tends to be worse 10- Escort EI A variety of appearances of malignant melanoma in the , ., . • J-. ,. , . , , head: a review. Radiographics 2001; 21: 625- 39. because the tumor is often discovered at a more advanced x L L e e S R s h i m i z u KT> ^ ^ et a l M u c o s a l m e l a n o m a o f t h e h e a d Stage ( 11). The prognosis seems to be related to the extent and neck: the impact of local control on survival. Laryngoscope and location of the primary tumor and the adequacy of the 1994; 104: 121- 6. 1 • • Aiii. 1. 4.1. c • 1 4 12. Sabel MS, Sondak VK. Pros and cons of adjuvant interferon in the surgical excision. Although the 5- year survival rate ranges . ,_ . , , „ , . ( , m , „ . J t1 „ 0 o J o treatment of melanoma. Oncologist 2003; 8: 451- 8. from 14% to 4 5% for patients with head Or neck mucosal 13. Harkness KA, Manford MR. Metastatic malignant melanoma melanoma ( 2,9,11), the 5- year survival rate was 0% for presenting as a cavernous sinus syndrome. J Neurol 2004; 251: 224- 5 patients with sinus melanoma in one report ( 2,13- 15). .. , T '. „ , r ,... .. c , , „ . , . . ,. . , r c • ' 14. Nogami K, Nishijima M, Endoh S, et al. Malignant melanoma RFFFRFNPFS metastatic to the cavernous sinus and skull with an unknown primary origin: report of a case. No Shinkei Geka 1992; 20: 1017- 20. 1. Batsakis JG, Regezi JA, Solomon AR, et al. The pathology of head 15. Hufnagel TJ, Savino PJ, Zimmerman RA, et al. Painful ophthalmo-and neck tumors: mucosal melanomas, part 13. Head Neck Surg plegia caused by neurotropic malignant melanoma. Can J Ophthalmol 1982; 4: 404- 18. 1990; 25: 38^ 1. |
