2019 Research Forum

Thy Nguyen MS IV, Maryam Talai-Shahir MD R3, Arash Heidari MD Department of Medicine, Kern Medical, Bakersfield , CA, United States HELIOTROPE RASH PRECLUDING METASTATIC OVARIAN CANCER

DISCUSSION In up to 20% of cases reported, dermatomyositis appears as a paraneoplastic syndrome associated with multiple malignancies such as ovarian, breast, prostate, lung, nasopharyngeal and colorectal cancer, and non-Hodgkin lymphomas. It can be presented either before, in the time, or after cancer diagnosis. The association may be higher in those aged 40 years or older. Patients with dermatomyositis are advised to undergo malignancy screening during the first 3 years after the diagnosis of dermatomyositis, except for ovarian cancer that may present even 5 years following the diagnosis of dermatomyositis. Thus, patients are advised to Ca-125 testing every 6 months; however, screening for ovarian cancer with Ca-125 in dermatomyositis has a sensitivity of 50%. CONCLUSION Dermatomyositis as a paraneoplastic phenomenon in ovarian cancer has been reported. The diagnosis should be highly suspected based on clinical findings, despite inconclusive immunological findings. The interval from recognition of dermatomyositis to development of underlying malignancy is variable but often at time of diagnosis or shortly thereafter. Thus patients should receive initial malignancy evaluation and yearly surveillance for malignancy screening to aid in early detection and management. REFERENCES 1. Abu-Yousef, M, Pelsang, R, et al , Glob. libr. women's med . (ISSN: 1756-2228) 2008; DOI 10.3843/GLOWM.10266

INTRODUCTION

INTERVAL FINDING

Dermatomyositis is a chronic inflammatory disease of the muscle and skin. The disease is rare, incidence of 0.5–0.89 per 100,000, female to male predominance 2:1. The cardinal symptom is a skin rash that precedes or accompanies progressive muscle weakness. Because ovarian cancer is not a common presentation in women with dermatomyositis, it makes malignancy surveillance a challenged diagnosis. A 61-year-old Cambodian female presented to emergency department with subjective fever and sharp, non-radiating right eye pain for three days. No associated neuropathies or muscle pain. On examination, patient was afebrile, hemodynamically stable, a circumscribed raised placoid lesion noted on right medial eye, erythematous excoriation of upper right eyelid consistent with heliotrope rash . There was also dry scaly rash with lichenification involving both ears and left upper lip. Musculoskeletal and neurological examination was normal. CASE PRESENTATION

T wo years later, the patient presented to emergency department with complain of severe diffuse abdominal pain, intractable nausea/vomiting, and heavy postmenopausal vaginal bleeding. Repeat CT chest/abdomen/pelvis with contrast showed 10 cm right ovarian mass. Lab finding significant for elevated CA-125 of 1661U/ml. Patient was diagnosed with stage IV ovarian carcinoma and stage IB1 squamous cell carcinoma of the cervix and initiated chemotherapy. Now the patient is status post total hysterectomy, bilateral salpingo-oophorectomy, lymphadenectomy, and sigmoidectomy with anastomosis. A 19.9 cm mass was resected from the left ovary; a resected 10mm renal lymph node was positive for malignancy. Pelvic washing cytology indicated diffuse malignant clusters. Patient was started on Carboplatin AUC 6 and Paclitaxel 175 mg/m2 of 6 cycle total. Most recent CA-125 of 23.

Figure: Pap smear revealed atypical glandular cells with small eccentric hyperchromatic nuclei and clean background.

COMPUTED TOMOGRAPHY SCAN OF ABDOMEN & PELVIS

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(2) Figure: (A) Initial CT pelvis with IV contrast revealed normal bilateral ovaries (B) Repeated CT scan 2 years later revealed 10 cm right ovarian mass (1)

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Figure: (1) Gottron papules seen in dermatomyositis of the hand in patient with similar condition Initial lab work ruled out infectious, immunological, oncological etiology. CT chest/abdomen/pelvis with contrast and colonoscopy completed showed no underlying malignancy. She was prescribed steroid cream, which helped improve the rash and to follow-up outpatient but was lost to follow-up.

2. Arshad Ilyas, Barton Desmond. Dermatomyositis as a paraneoplastic phenomenon in ovarian cancer. BMJ Case Rep. Aug. 2016.

3. Carlie Field and Barbara A. Golf. Case Report: Dermatomyositis, key to diagnosing ovarian cancer, monitoring treatment and detecting recurrent disease. Gynecologic Oncology Reports. Nov., 2017.

4. Dourmishev L, Dourmishev A. Dermatomyositis: advances in recognition, understanding and management. Springer-Verlag, 2009.

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5. Pannu HK, Corl FM, Fishman EK. CT Evaluation of Cervical Cancer: Spectrum of Disease. Radiographics Sep 2011 Vol. 21, No. 5

6. Whitmore SE, Anhalt GJ, Provost TT et al. Serum CA-125 screening for ovarian cancer in patients with dermatomyositis. Gynecol Oncol 1997;65:241–4

Figure: (A) CT pelvis with IV contrast at the initial presentation (B) Repeated CT pelvis 2 years later revealed lobulated heterogeneously enhancing lesion measuring 12.2 x 11.6 x 10.1 cm in the pelvis

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