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Case Presentation Summer Pathology Seminar IAP 2012 Senec, Slovak Republic June 8-9, 2012

Case Presentation Summer Pathology Seminar IAP 2012 Senec, Slovak Republic June 8-9, 2012. Fredrik Petersson MD, PhD Associate Professor Senior Consultant Department of Pathology National University of Singapore National University Health System. Clinical History.

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Case Presentation Summer Pathology Seminar IAP 2012 Senec, Slovak Republic June 8-9, 2012

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  1. Case PresentationSummer Pathology Seminar IAP 2012 Senec, Slovak Republic June 8-9, 2012 Fredrik Petersson MD, PhD Associate Professor Senior Consultant Department of Pathology National University of Singapore National University Health System

  2. Clinical History A 54 year old Chinese man developed progressively worsening stridor over the course of a few months. A CT scan revealed an subglottic laryngeal lesion measuring 1.5 cm located just below the vocal cords with partial obstruction of the lumen. A biopsy of the lesion was performed reported as a “soft tissue sarcoma with smooth muscle differentiation” (not by me). Pre-operative chest x-rays, ultra-sound examination of the abdomen and MRI of the brain did not show any concurrent tumors. A total laryngectomy was performed.

  3. 2. Divider Introducing new topic Histology

  4. Lymph node Tumor Cricoid Tumor Tumor composed of two different components

  5. Well circumscribed, unencapsulated

  6. Slit-like blood vessels

  7. Fascicles of spindle cells with bland nuclear features

  8. Two sharply separated components

  9. Cellular component – primitive appearing round cells

  10. Focal nuclear atypia

  11. Light Microscopic Features - Summary Biphasic non-encapsulated, well circumscribed tumor with no invasion into cartilage. (1) Moderately cellular spindle cell component with no significant nuclear atypia or mitotic activity. Presence of blood vessels. (2) A primitive, small round cell component featuring focal nuclear atypia (and mitotic activity). (1) and (2) sharply separated.

  12. 3. Divider Introducing new topic Immunohistochemistry

  13. SMA positive in both components

  14. SMA spindle cells

  15. SMA

  16. SMA round cell component

  17. SMA

  18. Desmin

  19. CD34 highlighted abundant vessels also in the cellular component

  20. CD34 – spindle cell component

  21. CD3 – Intratumoral T-cells

  22. CD3

  23. Immunohistochemistry - Summary Both components strongly positive for SMA and negative for desmin. Prominent vasculature (also in the primitive small round cell component). Intratumoral T-cells. Negative for S100 protein, CD34 and cytokeratins.

  24. Diagnosis?

  25. Additional History The patient had developed chronic renal failure and had a renal transplant 14 years prior to the development of the laryngeal tumor. The patient was on an immunosuppressive drugs; cyclosporine A and azathioprine.

  26. Magic Bullet…

  27. EBV (EBER) in situ hybridization

  28. EBER – strongly positive in both components

  29. EBER

  30. EBER

  31. EBER

  32. Diagnosis: EBV associated Smooth Muscle Tumor

  33. I found that the patient had a previously diagnosed cutaneous angioleiomyoma. Which I retrieved and reviewed. Working Up the Case…..

  34. Fascicles of bland myoid cells

  35. Medium sized blood vessels

  36. Fascicles of myoid cells blending imperceptively with blood vessel

  37. Morphology consistent withAngioleiomyoma/Vascular Leiomyoma But…..

  38. EBV (EBER) ISH – strongly positive

  39. EBV associated SMT Revised DX

  40. EBV SMT - History In 1970 and 1971, smooth muscle tumors associated with immunosuppression were first reported. In the 1980s, there were steadily increasing numbers of patients with AIDS and transplant related immunosuppression and smooth muscle tumors, variably considered as leiomyomas or leiomyosarcomas. In 1994/95 the causative link between these tumors and Epstein-Barr virus was established.* Have also been associated with autoimmune diseases and common variable immunodeficiency syndrome . * McClain KL et al. N Engl J Med. 1995 Jan 5;332(1):12-8. * Prevot S et al. Virchows Arch. 1994;425(3):321-5.

  41. EBV SMT – Anatomical Distribution • EBV-SMTs have been described in a wide variety of anatomical locations and are frequently multifocal. • Liver, spleen, gall bladder, tonsils, palate, nasopharynx, skin, pharynx, cribriform plate, orbita, larynx, mesentery, lung, iris, bone, skin, brain, meninges, spinal cord, common bile duct, myocardium, lymph nodes, adrenals, breast, pleura, urinary bladder, stomach, duodenum, gallbladder, spleen, small bowel, pericardium and bronchi.

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