SOFT TISSUE SARCOMAS WITH EPITHELIOID MORPHOLOGY Îà¾åÈé·ÁÂÖ¤ò¼¨¤¹ÆðÉô¼ðáç*1 †
Angelo P. Dei Tos M.D. Dep. of Pathology& Oncology Treviso, Italy --International Seminar at Lake Hamana, 2004¤Î¹Ö±é¤è¤ê
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Contents:
Îà¾åÈéÆù¼ð epithelioid sarcoma †
Îà¾åÈé´ÖÍշϼðáç¤ÎÂåɽŪ¼ðáç¤Ç, 1970ǯEnzinger¤Ë¤è¤ê»Ï¤á¤ÆÊó¹ð¤µ¤ì¤¿¡£
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1. Classic necrobiotic(granulomatous)growth pattern
- coalescent cystic nodules lined by festoons composed of polygonal and spindle cells
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- ¾åÈéÍͺÙ˦¤ÎÂçÉôʬ¤ÏÃæ¿´Éô¤Ë°ÌÃÖ¤·Ë¿î·ÁºÙ˦À®Ê¬¤Ï·ëÀá¼þÊÕÉô¤ËÊкߤ¹¤ë¤¬, ξ¼Ô¤Ï¼¡Âè¤Ë°Ü¹Ô¤·¤Æ¤¤¤ë¡£³Ë¤Ï±ß·Á, vesicular¤Ç¾®·¿¤Î³Ë¾®ÂΤòͤ¹¤ë¡£Â¿·¿À¤ÏÄ̾老¤¯¤ï¤º¤«¡£
2. Fibroma-like variant
Ë¿î·ÁºÙ˦¤¬Í¥°Ì¤Ç, ¾åÈéÍͺÙ˦¤ÏÌÜΩ¤¿¤Ê¤¤¡£ËÉÙ¤Ê籸¶Àþ°Ý´Ö¼ÁÆâ¤Ë¤ï¤º¤«¤Ê°Û·¿¤ò¤·¤á¤¹ºÙ˦¤¬Â¸ºß¤¹¤ë¡£¿ÇÃǤˤÏÌȱÖÀ÷¿§(cytokeratinÍÛÀ)¤¬Èó¾ï¤Ë͸ú¤È¤Ê¤ë¡£
3. Angiomatoid variant
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ES classic necrobiotic variant¤Ï´Ä¾õÆù²ê¼ð, ¥ê¥¦¥Þ¥Á·ëÀ᤬´ÕÊ̤Ȥʤ뤬, ¤³¤ì¤é¤Ïkeratin±¢À, CD68ÍÛÀ¤«¤é¿ÇÃDzÄǽ¤È¤Ê¤ë¡£
4. Proximal-type/ variant
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- ³êËìÆù¼ð
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Îà¾åÈé·ì´ÉÆâÈé¼ð epithelioid hemangioendothelioma(EHE) †
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agressive¤ÊEHE †
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- EHE¤Ï½½Ê¬¤Ë°À¼ðáç¤È¤ß¤Ê¤¹¤Ù¤¤Ç¤¢¤ë(2002ǯWHOʬÎà)
EHE¤ÎES-like variant. †
- CD34¤¬±¢À¤Î¤¿¤á, HEÁÈ¿¥¤Ç¤Î´ÕÊÌ¿ÇÃǤ¬º¤Æñ¤È¤Ê¤Ã¤Æ¤¤¤ë¡£
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Îà¾åÈé·ì´ÉÆù¼ð epithelioid angiosarcoma †
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- ÇÙ, Éû¿Õ, ¹Ã¾õÁ£¤ÏÈæ³ÓŪÉÑÈˤËǧ¤á¤é¤ì¤ë¡£
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- ±ó³Öž°Ü¤ÏÇÙ, ¥ê¥ó¥ÑÀá, ¹ü, ÆðÉôÁÈ¿¥¤ËÉÑÅÙ¤¬¹â¤¤¡£
- ·ÁÂÖÁü, cytokeratin¤Îȯ¸½¤Ë¤ª¤¤¤ÆÎà¾åÈéÆù¼ðES¤Îangiomatoid variant¤È¤Î´ÕÊ̤¬½ÅÍפȤʤ롣Ëܼðáç¤Ï¤è¤ê¿·¿À¤¬¶¯¤¯, Ä̾ïCD31, factor¼¤òȯ¸½¤·¤Æ¤¤¤ë¤³¤È¤Ç´ÕÊ̤¹¤ë¡£
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Îà¾åÈé·¿°ÀËö¾¿¿À·Ð¾ä¼ðáç †
- °ÀËö¾¿¿À·Ð¾ä¼ðáç(MPNST)¤Ï¼ï¡¹¤Îʬ²½¤òÍ褹¤³¤È¤¬¤¢¤ê, ¤³¤ì¤òÇØ·Ê¤Ë, Îà¾åÈé·ÁÂÖ¤ò¤È¤ë¤³¤È¤¬¤¢¤ê¤¦¤ë. Á£Í͹½Â¤¤ò¤È¤ë¤³¤È¤¬¤¢¤ê, keratin¤äEMA¤¬ÍÛÀ¤Ë¤Ê¤Ã¤¿¤ê, ¤·¤Ð¤·¤Ðchromogranin¤äsynaptophysin¤Ê¤É¿À·ÐÆâʬÈç¥Þ¡¼¥«¤òȯ¸½¤·¤¿¤ê¤¹¤ë.
- ¼ï¡¹¤Îʬ²½¤òȼ¤¦MPNST¤ÎȾ¿ô¤ÏNF1 syndrome¤È´ØÏ¢¤·¤Æ¤ª¤ê, ´ÕÊ̤˰äÅÁ³ØÅª¸¡ºº¤¬ÍÍѤʤ³¤È¤¬¤¢¤ë.¤³¤ì¤é¤Î¾ÉÎã¤Ç¤ÏÁ´Î㤬³êËìÆù¼ð ¤ËÆÃ°ÛŪ¤Êt(X;18)žºÂ¤¬·çÇ¡¤·¤Æ¤¤¤ë¤³¤È¤¬³Îǧ¤µ¤ì¤Æ¤¤¤ë¡£
- ¤Þ¤ì¤ËMPNST¤ÏÁ´Îΰ褬¾åÈéÍÍ·ÁÂÖ¤ò¼¨¤¹¤³¤È¤¬¤¢¤ë¡£¤³¤ì¤ÏÎ×¾²ÉÂÍý³ØÅª¤ËÌÀ³Î¤Ë¶èÊ̤µ¤ì¤ë¼À´µ¤Î²ÄǽÀ¤¬¤¢¤ë.
Îà¾åÈé·¿MPNST †
˦Áã·¿ÆðÉôÆù¼ð Alveolar soft parts sarcoma(ASPS) †
¿Õ³°¥é¥Ö¥É¥¤¥É¼ðáç extrarenal rhabdoid tumor (ERT) †
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