Ovarian cancer and dermatomyositis

1. Ovarian cancer and dermatomyositis Department of Oncology,Gynecology Kaplan Medical Center

2. C a s e r e p o r t A 69-year –old woman was admitted on July 2000 to the gynecological department with abdominal pain , distention ,dyspnea and weightloss for investigation.

3. C a s e r e p o r t From her previous medical history: Well controlled HTN Osteoporosis Vitiligo Gynecological status: G-11,P-3,A-8 Menopausal since age of 43 No family history for malignancy

4. E v a l u a t i o n CT-scan of chest and abdomen- pleural effusion and ascites, huge pelvic and ovarian masses, omental cake. Marked CA-125 elevation-1744. Cytological examination of ascitic fluid- malignant epithelial cells

5. Diagnosis of ovarian cancerwas made and treatment was started

6. T r e a t m e n t On August 2000 - first course of chemotherapy Carboplatinum and Paclitaxel. Seven days later a shawl-like, erythematous rash over upper chest, back and forehead appeared. Dermatologist consultation: DD: Photosensitivity drug eruption Paraneoplastic syndrom: dermatomyositis Treatment with prednisone and cidaline no response

7. C a s e r e p o r t A few days later she complained of difficulty climbing stairs and muscle soreness. Examination: Proximal muscle weakness CPK:1148 Muscle biopsy was performed: features consistent with a histologic diagnosis of dermatomyositis

8. After two additional courses ofchemotherapy: Severe dysphagia, hoarseness, proximal muscle weakness , neutropenia, severe exacerbation of skin rash with vesicle formation. Laboratory findings: CPK-(357), CA-125-( 62) No symptomatic improvement with steroids IVIG –treatment was started

9. After 3 additional courses ofchemotherapy with dose reduction: Complete CA-125 normalization – 15( N<30) Complete disappearance of dermatomyositis CT-scan normal Pt referred for explorative laparotomy – Surgical debulking On January 2001 pt was operated: TAH+BSO +Infracolic omentectomy –optimal debulking performed.

10. Pathological examination Both ovaries – no tumor seen. Omentum: moderately to poorly differentiated adenocarcinoma Uterus and fallopian tubes –no tumor seen. Pt was placed on close follow-up with CA-125- marker detection, physical examination and serial CT-scan every 3 months

11. C a s e r e p o r t Four mo later- marker relapse-CA-125- (50) Severe exacerbation of skin rash Recurrent ascites- not massive Chemo with Taxotere+Carboplatinum started. After four courses CA-125 declined not significantly as well as skin rash.

12. C a s e r e p o r t Despite the treatment disease progressed Pt was switched to the third line of chemotherapy with Gemcitabine No biochemical response was achieved Radiologically- slow progression Dermatomyositis partially controlled with immunosupressant and IVIG therapy

13. Risk of cancer in pts with DM andfollow-up implications:a Scottish population-based cohort study BrJ Cancer 2001 Jul 6, Stockton D, Doherty VR, Brewter Retrospective population-based cohort study-705 pts with DM 1982-1996. Malignancy was diagnosed in 50 pts with DM (SIR 7.7, 95%CI 5.7-10.1). Risk was elevated for both sexes ,significantly for females, highest in pts aged 45-74 at the time of diagnosis for DM

14. First case described-in 1916 Williams –1959-590 cases of DM –15% tumor rate-most with skin lesions. DM associated with a greater frequency of malignancy than in the general population Among gynecological malignancies ,coexistence of ovarian cancer and DM is most frequent. DM can be the only presenting symptom of ovarian cancer-evaluation for suspected malignancy should be done DM-rare paraneoplastic syndromthat may precede malignancy by several months

15. I n c i d e n c e Ovarian cancer occurred in 13.3% of the total femalepopulation, a much higherthan the 1% observed in the general population, and in 21,4% of females aged over 40with DM.

16. S c r e e n i n g Serum CA-125 screening for ovarian cancer in pts with dermatomyositis. Whitmore SE, …( The Johns Hopkins University,Baltimore) Gynecol Oncol. 1997 May,65 A single blinded ,case-control study in 14 women with DM between 1986-1993. Four pts–developed ovarian cancer. Sensitivity of CA-125 elevation for detection OC was 50%, specificity was 100%. Prospective studies are needed

17. Most common symptoms Rash- shawl –like distribution,Gottrons papules, cuticular changes(periungal teleangiectasia,photo- distributed erythema or poikiloderma,scaly alopecia Proximal muscle weakness CPK- elevation Vesicle-formation-strongly related to the presence of malignancy,especially gynecological? (Kubo, Tamaki ,Japan) Cricopharyngeal achalasia? (Peters,Thornton)

18. T r e a t m e n t In most cases symptoms of DM regressed after treatment of the underlying carcinoma. Manifestations of DM progressed in synchrony with tumor spread. In the cases of progression of underlying malignancy corticosteroids and IVIG may be effective

19. C o n c l u s i o n s Ovarian cancer is the most overrepresented malignancy diagnosed in women with DM . In the most cases DM precedes the diagnosis of ovarian cancer,but it may also present simultaneously. Generally screening of ovarian cancer is not effective, but serum CA-125 detection in pts having DM may be useful. The development of DM after an established ds of ovarian cancer,as demonstrated by our pt ,seems to be less common Prognosis of such pts most probably is poor.