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Show Paraneoplastic Dermatomyositis Related to a Chondrosarcoma Involving the Cavernous Sinus Mrinali M. Patel, MD, Rebecca C. Stacy, MD, PhD Abstract: Approximately one third of all cases of dermato-myositis may be associated with malignancy. We describe a patient with unexplained rash, joint pain, and muscle weakness, who subsequently developed a cavernous sinus syndrome due to a central nervous system chondrosarco-ma. Discovery of this tumor and further dermatologic evaluation, including skin biopsy, resulted in diagnosis of paraneoplastic dermatomyositis due to cavernous sinus chondrosarcoma. Journal of Neuro-Ophthalmology 2013;33:363-366 doi: 10.1097/WNO.0b013e3182a30480 © 2013 by North American Neuro-Ophthalmology Society A51-year-old woman with a history of asthma presented with 1 month of painful, red skin eruptions of the face and buttocks and the dorsal and palmar surfaces of both hands (Fig. 1). A skin biopsy from a lesion on the left elbow was nondiagnostic, showing dermal fibrosis and minimal inflammation. She was treated with topical clobetasol and FIG. 1. Skin lesions characterized by red papules involving both hands, including the palmar surface and preferentially located in the interphalangeal areas. Department of Ophthalmology (MMP, RCS), Massachusetts Eye and Ear Infirmary, Harvard Medical School, Boston, Massachusetts. The authors report no conflicts of interest. Address correspondence to Rebecca C. Stacy, MD, PhD, Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, 243 Charles Street, Boston, MA 02114; E-mail: rebecca_stacy@meei.harvard.edu Patel and Stacy: J Neuro-Ophthalmol 2013; 33: 363-366 363 Photo Essay Section Editor: Timothy J. McCulley, MD Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. oral fluconazole and valacyclovir, without improvement. Four weeks later, she noted joint pain and swelling in the hands, wrists, hips, and shoulders, as well as weakness of her upper legs. An echocardiogram was negative for endocarditis. Laboratory studies included hemoglobin of 12.5 g/dL (normal: 12.0-16 g/dL), hematocrit of 35.9% (normal: 36%-46%), erythrocyte sedimentation rate of 100 mm/h, C-reactive pro-tein of 21.7 mg/L (normal: ,8.0 mg/L), ferritin level of 1,177 ng/mL (normal: 10-200 ng/mL), aspartate aminotransami-nase of 7 U/L (normal: 9-32 U/L), alanine aminotransferase of 87 U/L (normal: 7 - 30 U/L), aldolase of 10.9 U/L (normal: ,7.7 U/L), angiotensin-converting enzyme of 59 U/L (nor-mal: ,53 U/L), and antinuclear antibody positive at 1:40 (speckled). Other tests were normal, including rheumatoid factor, anti-nuclear cytoplasmic antibody, immunoglobulin and complement levels, hepatitis markers, Lyme, and parvo-virus. Electromyography of the right thigh showed active denervation in the right iliopsoas muscle without evidence of a peripheral neuropathy or myopathy. Treatment with 10 mg of prednisone lessened her joint pain, skin discontent, and proximal leg weakness. Six weeks later, the patient reported intermittent vertical diplopia. Neuro-ophthalmic examination revealed visual acuities of 20/20 in each eye with an intact afferent visual system, including funduscopy. Pupils were equal in size and reacted normally. There was 2 mm of right ptosis. Ocular motility testing revealed limited supraduction and abduc-tion of the right eye, and there was decreased sensation of the trigeminal and maxillary division of the right trigeminal nerve. Magnetic resonance imaging of the brain showed an enhancing mass (5.5 · 4.8 · 5.5 cm3) in the right petroclival FIG. 2. Postcontrast T1 axial (A) and coronal (B) magnetic resonance imaging reveals a mass in the right petroclival fissure, involving the cavernous sinus extending into the middle and posterior cranial fossae. FIG. 3. Skin biopsy. A. Focal vacuolar interface dermatitis, with lymphocytes infiltrating the junction between the epidermis and the dermis, causing damage to keratinocytes at the basal layer (hematoxylin-eosin, ·100). B. Higher magnification of area of basal keratinocyte damage and resulting vacuolar change. Dying keratinocytes appear bright pink (arrow) (hema-toxylin- eosin, ·200). C. There is thickening of the basement membrane of the epidermis (arrows), a sign of previous damage (periodic acid-Schiff, ·100). D. Colloidal iron staining (blue) reveals mucin between collagen fibers, reflecting dermal mucinosis (mucicarmine, ·200). 364 Patel and Stacy: J Neuro-Ophthalmol 2013; 33: 363-366 Photo Essay Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. fissure involving the right cavernous sinus (Fig. 2). A second skin biopsy from the dorsum of the left hand demonstrated a combination of vacuolar interface dermatitis and dermal mucinosis consistent with dermatomyositis, lupus, or para-neoplastic dermatomyositis (Fig. 3). Given the presence of a large central nervous system tumor, paraneoplastic dermatomyositis was thought to be the most likely diagnosis. Computed tomography of the chest, abdomen, and pelvis failed to disclose any other source of malignancy. A craniotomy for partial debulking and biopsy of the tumor was performed, revealing a myxoid chondro-sarcoma, Grade II (Fig. 4A, B). Consistent with this diagno-sis, immunostaining was positive for S100 (Fig. 4C) and negative for epithelial marker antigen and pancytokeratin. The patient's prednisone dose was increased to 20 mg/ day, and 6 weeks later, her diplopia resolved in primary gaze, with 1 mm of right ptosis and only mild limitation of abduction of the right eye. She also reported further improvement in her joint pain but still had persistent skin lesions and was transitioned from steroids to mycophenolate mofetil. She began adjuvant radiation therapy for her chon-drosarcoma 4 months later. Her diplopia did not recur during a follow-up of 1 year. Thirty percent of dermatomyositis cases are due to an underlying malignancy, but the majority of these are pulmonary or gynecological in origin (1,2). To our knowl-edge, there are no previous reports of a cavernous sinus tumor initially presenting with paraneoplastic dermatomyositis. Moreover, there is only 1 previous report of dermatomyositis secondary to chondrosarcoma that involved the tibia (3). Dermatomyositis is a cutaneous disease with a myriad of skin findings, most classically Gottron papules of the interphalangeal areas of the fingers, or a heliotropic rash of the eyelids and cheeks. Proximal muscle weakness often is present (4). Laboratory findings for typically include elevated transaminases, aldolase, and creatinine kinase and the pres-ence of anti-Mi-2 or anti-Jo antibodies (4). Normal creati-nine kinase (5) and absence of anti-Mi2 or anti-Jo antibodies (6) may be more typical of paraneoplastic dermatomyositis and should prompt systemic workup for malignancy. Testing for additional antibodies, such as CA125, for ovarian tumors may be considered. An antibody that seems to be specific for cancer-associated dermatomyositis ("anti-155/140") has been reported (7) but is not yet commercially available. Our patient presented with skin eruptions, joint pain, and proximal muscle weakness. The cause of her signs and symptoms was not detected for 6 weeks until she underwent imaging for a right cavernous sinus syndrome, which revealed her malignancy. Laboratory tests for anti-Mi-2 and anti-Jo were negative, consistent with paraneoplastic dermatomyosi-tis. Despite successful partial resection of her tumor, portions of her chondrosarcoma remained unresectable. This may explain the persistence of her skin lesions following surgery. REFERENCES 1. Callen JP. Dermatomyositis and malignancy. Clin Dermatol. 1993;11:61-65. 2. Stockton D, Doherty VR, Brewster DH. Risk of cancer in patients with dermatomyositis or polymyositis, and follow-up implications: a Scottish population-based cohort study. Br J Cancer 2001;85:41-45. FIG. 4. Chondrosarcoma. A. Multilobular architecture of chondrosarcoma (hematoxylin-eosin, ·40). B. The tumor is comprised of small uniform cells with bland nuclei popu-lating a myxoid matrix (hematoxylin-eosin, ·200). C. Tumor cells exhibit positive nuclear staining for S100 (im-munoperioxidase stain, ·200). Patel and Stacy: J Neuro-Ophthalmol 2013; 33: 363-366 365 Photo Essay Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. 3. Mol MJ, Stalenhoef AF, Boerbooms AM. Chondrosarcoma coexistent with dermatomyositis. Arthritis Rheum. 1986;29:813-814. 4. Khan S, Christopher-Stine L. Polymyositis, dermatomyositis, and autoimmune necrotizing myopathy: clinical features. Rheum Dis Clin North Am. 2011;37:143-158. 5. Fudman EJ, Schnitzer TJ. Dermatomyositis without creatinine kinase elevation. Am J Med. 1986;80:329-332. 6. Love LA, Leff RL, Fraser DD, Targoff IN, Dalakas M, Plotz PH, Miller FW. A new approach to the classification of idiopathic inflammatory myopathy: myositis-specific autoantibodies define useful homogenous patient groups. Medicine. 1991;70:360- 374. 7. Madan V, Chinoy H, Griffiths CEM, Cooper RG. Defining cancer risk in dermatomyositis, Part II: assessing diagnostic usefulness of myositis serology. Clin Exp Dermatol. 2009;34:561-565. 366 Patel and Stacy: J Neuro-Ophthalmol 2013; 33: 363-366 Photo Essay Copyright © North American Neuro-Ophthalmology Society. Unauthorized reproduction of this article is prohibited. |
