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

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1. Classic necrobiotic(granulomatous)growth pattern

  • coalescent cystic nodules lined by festoons composed of polygonal and spindle cells ¿³Ñ¤¢¤ë¤¤¤ÏË¿î·ÁºÙ˦¤«¤é¤Ê¤ë²ÖÌÖ(festoon)¹½Â¤¤Ë¤è¤ê±ï¼è¤é¤ì¤¿Ç¹Ë¦¾õ·ëÀá¤ÎÌþ¹ç
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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®ÂΤòÍ­¤¹¤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¤ËÍ­¸ú¤È¤Ê¤ë¡£

3. Angiomatoid variant
ºÇ¤â¤Þ¤ì¤ÊES¤Îvariant¤Ç¤¹¡£ÁÈ¿¥¤Ç¤Ï¾åÈéÍͺÙ˦, Ë¿î·ÁºÙ˦¤«¤é¤Ê¤ëÊɤȷì±Õ¤Ë½¼¤¿¤µ¤ì¤¿Ç¹Ë¦À­¼ðáî¡£ÆâÈé¤Î¥Þ¡¼¥«¤¬±¢À­¤Ç¤¢¤ë¤³¤È¤«¤éÎà¾åÈé·ì´ÉÆù¼ð¤È´ÕÊ̤µ¤ì¤ë¡£
CD34+¤È¤Ê¤ë¤³¤È¤¬¤¢¤êangiosarcoma¤È´Ö°ã¤¨¤ë¤¬CD31¤Ïɬ¤º±¢À­.

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4. Proximal-type/ variant
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¼ðáçºÙ˦¤Ïsmooth muscle actin¤ËÍÛÀ­¡£desmin¤Ï±¢À­¡£

¥é¥Ö¥É¥¤¥ÉºÙ˦¡§ES proximal variant¤Ë¤è¤¯½Ð¸½¤¹¤ë¤¬, ¤³¤Î°¡·¿¤ËÆÃ°ÛŪ¤Ç¤Ï¤Ê¤¤¡£¤³¤ÎºÙ˦¤Ïͽ¸å¤Î°­¤¤¼ï¡¹¤Ê¾ÉÎã¤Ë¸«¤é¤ì¤ë¤Î¤Ç, ñ¤Ê¤ëæʬ²½¤ò¤¢¤é¤ï¤·¤Æ¤¤¤ë¤Î¤«¤â¤·¤ì¤Ê¤¤¡£

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  • ³êËìÆù¼ð
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  • ¿Õ³°¥é¥Ö¥É¥¤¥É¼ðáç extra-renal rhabdoid tumor(ERT) proximal type ES¤Î¼çÍפʴÕÊ̼À´µ¤È¤Ê¤ë¡£¥é¥Ö¥É¥¤¥É¤Î½ê¸«¤¬¼çÂΤξì¹ç¤ÏHEÁÈ¿¥¤Ç¤Î´ÕÊ̤ϤۤÜÉÔ²Äǽ¡£ERT¤ÏÆýÍÄ»ù¤Ëȯ¾É¤·Èó¾ï¤Ëagressive¤Ê·Ð²á¤ò¤¿¤É¤ë¡£

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¹Å²½À­Îà¾åÈéÀþ°ÝÆù¼ð¡¡Sclerosing epithelioid fibrosarcoma (SEF)

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Îà¾åÈé·ì´É·Ï¼ðáç

Îà¾åÈé·ì´ÉÆâÈé¼ð epithelioid hemangioendothelioma(EHE)

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  • EHE¤ÎÉÂÊѤϤ褯, °ìËܤηì´É¤«¤éÉÂÊѤ¬È¯À¸¤¹¤ë¤è¤¦¤Ë»×¤ï¤ì¤ë
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  • ÌȱÖÁÈ¿¥À÷¿§¤Ç¤Ï·ì´ÉÆâÈé¥Þ¡¼¥«¤Îfactor­¼´ØÏ¢¹³¸¶, CD31, CD34, ³ËÆâž¼Ì°ø»Ò¤ÎFLI-1¤¬Ä̾ïȯ¸½¤·¤Æ¤¤¤ë¡£

agressive¤ÊEHE

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  • Ä귿ŪEHE¤Ç¤Ïž°ÜΨ¤Ï20%°Ê²¼, »à˴Ψ¤ÏÌó3%¡£ÁÈ¿¥Áü¤Ë°Û·¿¤òǧ¤á¤ë¾ÉÎã¤Ç¤Ï»à˴Ψ¤Ï20%¤ËÁý²Ã¤¹¤ë¡£
  • 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¡£
  • cytokeratin, CD31, FLI-1¤Ï¤¤¤Ä¤âÍÛÀ­¤È¤Ê¤ê, ES¤È¤Î´ÕÊ̤ËÌȱÖÀ÷¿§¤¬½ÅÍפȤʤäƤ¤¤ë¡£

Îà¾åÈé·ì´ÉÆù¼ð epithelioid angiosarcoma

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  • ¤Þ¤ì¤Ë´â¤ËÎà»÷¤¹¤ë½¼¼ÂÀ­Áý¿£¥Ñ¥¿¡¼¥ó¤¬¼çÂΤÎËܼðá礬¤¢¤ê, Ä̾ï·ì´ÉÆâÈé¥Þ¡¼¥«¤Ë²Ã¤¨¤ÆEMA¤äkeratin¤Ê¤É¤Î¾åÈé¥Þ¡¼¥«¤¬ÍÛÀ­¤ò¤·¤á¤¹¤¿¤á¿ÇÃǤΥԥåȥե©¡¼¥ë¤Ë¤Ê¤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)

Îà¾åÈ鷿¿·Á·¿»éËÃÆù¼ð


*1 Dei Tos AP, International Seminar at Lake Hamana, 2004
*2
*3 Meis-Kindblom JM et al., Am J Surg Pathol; 19: 979-993, 1995

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