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A Case of Myasthenia Gravis Combined with Mediastinal
Leiomyosarcoma and Stevens-Johnson Syndrome
Dong-Kuck Lee, Young-Mi Kweon
Department of Neurology, School of Medicine, Catholic University of Daegu, Korea We report a case of 36-year-old woman with myasthenia gravis (MG) combined with mediastinal leiomyosarcoma (LMS) and Stevens-Johnson syndrome (SJS). She was admitted to ICU with the symptoms of acute onset headache,diplopia, ptosis, dysphagia, general weakness, and respiratory difficulty for several days. Physical examination revealedtachypnea, decreased breath sounds and dullness to percussion in right lower chest. Neurologic examination showedptosis, diplopia, decreased gag reflexes, and generalized proximal weakness. Laboratory studies revealed increasedserum acetylcholine receptor antibodies and positive Tensilon test. Chest CT showed a huge mass in the right middlemediastium but no evidence of thymic enlargement. Mediastinal LMS was diagnosed by ultrasound-guided needle biop-sy. The myasthenic symptoms were fluctuated in spite og intravenous immunoglobulin, plasmapheresis, and corticos-teroid. During therapy, SJS developed. She died 4 months after the onset of the myasthenic symptoms despite thechemotherapy for LMS.
Key Words: Myasthenia Gravis, Mediastinal Leiomyosarcoma, Stevens-Johnson Syndrome
Address for correspondence
Dong Kuck Lee, M.D.
Department of Neurology, School of Medicine
Catholic University of Daegu
3056-6 Daemyung 4 Dong, Namgu, Daegu, 705-718, Korea
Tel: +82-53-650-4267 Fax: +82-53-654-9786
E-mail : [email protected]
Copyright 2004 by the Korean Society for Clinical Neurophysiology Figure 1. Chest CT shows a huge, well-enhanced mediastinal
Figure 2. Skin biopsy shows dermo-epidermal pustules (
tumor containing multiple nodular calcifications and abundant containing vacuolar degenerated cells, necrotic keratinocytes at ), and perivascular infiltration of lymphocytes in J Korean Society for Clinical Neurophysiology / Volume 6 / May, 2004 Figure 3. Histopathologic study shows bundles of malignant
smooth muscle fibers containing spindle-shaped nuclei (H&E,
100). Immunohistochemical stainings were positive for vimentin and SMA (alpha smooth muscle actin).
J Korean Society for Clinical Neurophysiology / Volume 6 / May, 2004 REFERENCES
11. Hasegeliüner A, Abali H, Engin H, Akyol A, Ruacan S, Tan E, Güllü I, Altundag K, Güler N. Myasthenia gravis and lymphoblastic lymphoma involving the thymus: a rare association. Leukemia and lymphoma 2001;42(3):527-31. 12. E r og˘lu A, Kürkçüog˘lu C, Karaog˘ l a n og˘lu N, Gürsan N.
Primary leimyosarcoma of the anterior mediastinum. Eur J Cardio-thoracic Surg 2002;21:943-45. 13. Hirano H, Kizaki T, Sashikata T, Maeda T, Yoshii Y.
Leiomyosarcoma arising from soft tissue tumor of the mediastinum. Med Electron Microsc 2003;36:52-58.
14. C o h e n L M , S k o p i c k i D K , H a r r i s t T J , C l a r k W H .
Noninfectious vesiculobullous and vesiculopustular dis- eases. In; Elder D. Lever’s Histopathology of the skin. 8th ed. New York: Lippincott Williams & Wilkins. 1997;239- 15. Konishi T, Yoshiyama Y, Takamori M, Yagi K, Mukai E, Saida T, The Japanese FK506 MG Study Group. Clinical study of FK506 in patients with myasthenia gravis. Muscle 16. Koç F, Yerdelen D, Sarica Y. Myasthenia gravis and inva- sive thymoma with multiple intracranial metastases. J J Korean Society for Clinical Neurophysiology / Volume 6 / May, 2004 12. Papatestas AE, Osserman KE, Kark AE. The relationship 17. Ritchie DS, Underhill C, Grigg AP. Aplastic anemia as a between the thymus and oncogenesis. A study of the inci- late complication of thymoma in remission. Eur J Haematol dence of non thymic malignancy in myasthenia gravis. Br 18. Tanakaya K, Konaga E, Takeuchi H, Yasui Y, Takeda A, 13. Sunderrajan EV, Luger AM, Rosenholtz MJ, Maltby JD.
Yonoki Y, Murakami I. Colon carcinoma after thymecto- Leiomyosarcoma in the mediastinum presenting as superi- my for myasthenia gravis: report of a case. Surg Today or vena cava syndrome. Cancer 1984;53:2553-6. 14. Odom RB, James WD, Berger TG. Andrew’s Diseases of 19. Abrey LE, Askanas V. Association of myasthenia gravis the skin. 9th ed. New York: W.B. Saunders Company, with extrathymic Hodgkin’s lymphoma: complete resolu- tion of myasthenic symptoms following antineoplastic 15. Chorzelski T, Hashimoto T, Maciejewska B, Amagai M, Anhalt GJ, Jablonska S. Paraneoplastic pemphigus associ- 10. Masaoka A, Yamakawa Y, Niwa H, Fukai I, Saito Y, ated with Castleman tumor, myasthenia gravis, and bron- Tokudome S. Thymectomy and malignancy. Eur J chiolitis obliterans. J Am Acad Dermatol 1 9 9 9 ; 4 1 : 3 9 3 - 4 0 0 .
Cardiothoracic Surg 1994;8:251-3.
16. Izumi Y, Kinoshita I, Kita Y, Toriyama F, Tanighuchi H, 11. Pan CC, Chen PC, Wang LS, Chi KH, Chiang H. Thymoma Motomura M, Yoshimura T. Myasthenia gravis with dif- is associated with an increased risk of second malignancy.
fuse alopecia areata and pemphigus foliaceus. J Neurol J Korean Society for Clinical Neurophysiology / Volume 6 / May, 2004

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