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Primary testicular lymphoma ¸¶È¯À­ÀºÁã¥ê¥ó¥Ñ¼ð

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âдݼðáç¤Î5%¤¬¥ê¥ó¥Ñ¼ð¤Ç, 50ºÐ°Ê¾å¤ÎÃËÀ­¤Îâдݼðáç¤ÇºÇ¤â¹âÉÑÅÙ. ¾®»ù¤Ï¤´¤¯¤Þ¤ì. Ê¿¶ÑǯÎð¤Ï50ºÐÂå¸åȾ¤è¤ê60ºÐÂå.

ξ¦âдݼðáç¤ÇºÇ¤â¹âÉÑÅ٤ʤΤ¬¥ê¥ó¥Ñ¼ð. HIVÍÛÀ­´µ¼Ô¤µ¤ó¤Ï¾¯¤Ê¤¤. âдݰ­À­¥ê¥ó¥Ñ¼ð¤ÎÁǰø¤Ï¤ï¤«¤Ã¤Æ¤¤¤Ê¤¤.

ŵ·¿Îã¤Ç¤Ï, ¹Å¤¤ÄˤߤΤʤ¤±¢Ç¹¼ðáî ¾¯¿ôÎã¤Ç¤Ï¡¢´µ¼Ô¤ËÁ´¿È¾É¾õ¤Þ¤¿¤Ï°Û¾ï¤Ê¿À·Ð³ØÅª½ê¸«¤Ê¤É¤ÎÀºÁã³°¾É¾õ¤¬¤¢¤ë.

ÀºÁã¾åÂΤËȯÀ¸¤¹¤ë¥ê¥ó¥Ñ¼ð¤ÏÀºÁã¥ê¥ó¥Ñ¼ð¤è¤ê¤â¾¯¤Ê¤¯¡¢Àºº÷¤ËȯÀ¸¤¹¤ë¥ê¥ó¥Ñ¼ð¤ÏÈó¾ï¤Ë¤Þ¤ì. ¤³¤ì¤é¤ÎÉô°Ì¤Î¥ê¥ó¥Ñ¼ð¤ÎÎ×¾²Åª¤ª¤è¤ÓÉÂÍý³ØÅªÆÃħ¤Ï¡¢ÀºÁã¥ê¥ó¥Ñ¼ð¤ÎÆÃħ¤ÈÎà»÷¤·¤Æ¤¤¤ë.

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¸¶È¯À­âдݥê¥ó¥Ñ¼ð¤Ï¸¶È¯À­ÂçǾ¥ê¥ó¥Ñ¼ð¤ÈƱÍͤËimmune-privileged(ÌÈ±ÖÆÃ¸¢)site¤ËȯÀ¸¤·, ·ì±Õ-âдݥХꥢ¤Ë¤è¤êÌȱÖÈ¿±þ¤è¤êËɸ椵¤ì¤Æ¤¤¤ë.*7

  • ÌÈ±ÖÆÃ¸¢¡Êimmune privilege¡Ë; Á´¿È¤ÎÌȱַϤ«¤é³ÖÀ䤵¤ì¤Æ¤¤¤ë¡¤Â¡´ïÆâ¤Ç¼«¸ÊÀ©¸æ¤µ¤ì¤Æ¤¤¤ë¤Ê¤É¤ÎÍýͳ¤Ë¤è¤êÌȱֱþÅú¤ä±ê¾ÉÈ¿±þ¤Ê¤É¤¬µ¯¤³¤ê¤Ë¤¯¤¤À­¼Á¤Î¤³¤È¡¥ÌÈ±ÖÆÃ¸¢¤¬¤¢¤ë¤È¡´ï°Ü¿¢¤ÎºÝ¤ËµñÀäÈ¿±þ¤¬µ¯¤³¤ê¤Ë¤¯¤¤¤³¤È¤«¤é°Ü¿¢À®¸ùΨ¤¬¹â¤¤¤È¤µ¤ì¤ë¡¥Ç¾¡¤´ã¡Ê³ÑËì¡Ë¡¤ÌÓȱ¡¤ÀºÁ㡤Êì¿Æ¤Î»ÒµÜÆâ¤ÎÂÛ»ù¤Ê¤É¤ËÌÈ±ÖÆÃ¸¢¤¬¤¢¤ë¤È¹Í¤¨¤é¤ì¤Æ¤¤¤ë¡¥*8

Æù´ã½ê¸«; âдÝŦ½Ð½Ñ¤ÎɸËܤǤÏø³ì¿§, ³¥¿§¤¢¤ë¤¤¤ÏÇò¿§¤Î¶­³¦ÌÀÎÆ¤Ê¼ðáî. ¿ô¥ß¥ê¤«¤é16cm¤Þ¤Ç¤Î¥µ¥¤¥º. 50%¤Î¾ÉÎã¤Ç¤Ï, ÇòËì(tunica albuginea)¤òÀüÄ̤·¤ÆÁý¿£¤·¤Æ¤¤¤ë. ¿¤¯¤Î¾ÉÎã¤ÇÀºÁã¾åÂΤ¬¿¯½±¤µ¤ì¤Æ¤¤¤ë. ¾®¿ôÎã¤ÇÀºº÷¤¬¿¯¤µ¤ì¤ë.

¸÷¸²½ê¸«; ¥ê¥ó¥Ñ¼ð¤Ë¤è¤ê, ¾¯¤Ê¤¯¤È¤â°ìÉô¤ÎÎΰè¤ÎÀººÙ´É¤¬¾Ã¼º¤¹¤ë. Ëö¾¿¤ÎÎΰè¤Ç¤ÏÀººÙ´É¤Î´Ö¼Á¤Ë¥ê¥ó¥Ñ¼ðºÙ˦¤¬¿»½á¤¹¤ë. ¤Û¤È¤ó¤É¤Î¾ì¹ç¡¢¼ðáçºÙ˦¤ÏÀººÙ´É¤Ë¿¯Æþ, ÀººÙ´É¤Î¼þÊÕ¤òÀê¤á¡¢À¸¿£ºÙ˦¤È¥»¥ë¥È¥êºÙ˦¤òÃæ±û¤ËÊѰ̤µ¤»¤ë¤«, ¤Þ¤¿¤ÏÀººÙ´É¤ò´°Á´¤ËÀêµò¤·¤Æ¤·¤Þ¤¦.

1/3¤Î¾ÉÎã¤Ç¤Ï¹Å²½Áü¤ò¼¨¤¹.

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  • primary testicular lymphoma¤Î¤Û¤È¤ó¤ÉÁ´¤Æ¤¬DLBCL¤ÎÁÈ¿¥·¿.¡¡Â¿¤¯¤Ï, centroblasts¤«¤é¤Ê¤ë¤¬, immunoblast¤ä¿ʬÍճˤò¤â¤Ä¥ê¥ó¥Ñµå¤«¤é¤Ê¤ëÎã¤â¤¢¤ë.
    °ìÉô¤ÎÎã¤Ç¤Ï, ¼ðáçÀ­ßÉ˦·ÁÀ®¤ò¤â¤ÄÎΰ褬¤ï¤º¤«¤Ëǧ¤á¤é¤ì¤ë.
     
  • ÌȱÖÀ÷¿§¤Ç¤Ï, 90%¶á¤¯¤¬non germinal-center B-cel¤Îphenotype¤Ë¤Ê¤ê, MIB-1 LI¤Ïhigh¤Ç¤¢¤ë. BCL2¤Ï¤·¤Ð¤·¤ÐÍÛÀ­¤È¤Ê¤ë¤¬BCL2¤ÎžºÂ¤ÏÄ̾ïǧ¤á¤é¤ì¤Ê¤¤.
     
  • ¥ê¥ó¥Ñ¼ðºÙ˦¤Ïpan B-cell¹³¸¶ÍÛÀ­. ¤Þ¤¿CD10, BCL6¤¬ÍÛÀ­(¤³¤ì¤Ç¤Ï, non GC¤È¤Ï¤¤¤¨¤Ê¤¤¤Î¤Ç¤Ï¡©)¤È¤Ê¤ë. ¤·¤«¤·Åµ·¿Îã¤Ï,BCL±¢À­. ¤·¤«¤·BCL2ÍÛÀ­¤Î¾ÉÎã¤â¿¤¯¤ß¤é¤ì¤ë. ¤½¤Î¾ì¹ç¤Ë¤â,BCL2°äÅÁ»Ò¤ÎºÆ¹½À®¤Ï¸¡½Ð¤µ¤ì¤Ê¤¤.*9
     
  • DLBCL¤Ï¹âÅÙ¤Ësomatic hypermutation¤ò¼¨¤·, HLAȯ¸½¤ò¾Ã¼º¤·¤Æ¤¤¤ë. ¤³¤Î¤¿¤á, ¤ª¤½¤é¤¯ÌȱֲóÈò¤ò½õŤ·¤Æ¤¤¤ë¤È¹Í¤¨¤é¤ì¤ë.
     
  • ¤³¤Î¥ê¥ó¥Ñ¼ð¤Ï NF¦ÊB¤ÈPI3K·ÐÏ©³èÀ­²½¤¬´ØÍ¿¤·¤Æ¤ª¤ê, ¥ê¥ó»À²½STAT3, chronic active B-cell signal¤¬¾åÊýÀ©¸æ¤µ¤ì¤Æ¤¤¤ë. CXCR4¤Ï²á¾êȯ¸½¤¹¤ë.
     
  • ¤Û¤È¤ó¤É¤ÎÎã¤Ç, MYD88ÊѰۤ¬¸¡½Ð¤µ¤ì¤ë. 21ºÐ¤«, ¤è¤ê¼ã¤¤´µ¼Ô¤µ¤ó¤Ç¤Ï, DLBCL¤¬Ã±ÆÈ¤ÇȯÀ¸¤¹¤ë¤³¤È¤Ï¤Þ¤ì.

¾®»ù¤ÎÀºÁã¥ê¥ó¥Ñ¼ð¤ÇºÇ¤â¿¤¤¤â¤Î¤Ï, Burkitt's lymphoma¤Ç, ŵ·¿Åª¤Êstarry skyÁÈ¿¥Áü¤ò¼¨¤¹.
primary testicular follicular lymphoma¤Ï¤Þ¤ì. ȯ¾ÉÎã¤Ç¤Ï, ¿¤¯¤¬¸Â¶ÉÀ­¤ÎßÉ˦À­¥ê¥ó¥Ñ¼ð, grade2¤¢¤ë¤¤¤Ï3a¤Ç¤¢¤ë.´öÎ㤫¤Ï, DLBCL¤Îminor component¤¬Ç§¤á¤é¤ì¤ë¤¬, minor component¤Î¸ºß¤Ïͽ¸åÉÔÎɤȤϴØÏ¢¤·¤Ê¤¤.

¤ï¤º¤«¤ÊÎã¤Ë¤ª¤¤¤Æ MALT lymphoma, peripheral T-cell lymphoma NOS, anaplastic large-cell lymphoma, T-lymphoblastic lymphoma¤¬Êó¹ð¤µ¤ì¤Æ¤¤¤ë.*10*11
(MALT lymphoma¤Ë¤Ä¤¤¤Æ¤Ï, high-grade lymphoma¤ÎÉôʬ¤ËMALT lymphomaÁÈ¿¥¤òǧ¤á¤ë¾ÉÎã¤Î¤è¤¦¤Ç¤¢¤ë*12*13)

1998ǯ¤Î²òÀϤǤÏ, ÀºÁãDLBCL¤Ï¥ê¥ó¥ÑÀáDLBCL¤È¤¤¤¯¤Ä¤«¤Îʬ»ÒÀ¸Êª³ØÅªÆÃħ¤¬°Û¤Ê¤ë¥µ¥Ö¥»¥Ã¥È¤Ç¤¢¤ê, ÁÈ¿¥³ØÅª¤ª¤è¤Óʬ»ÒÀ¸Êª³ØÅª½ê¸«¤¬MALT lymphoma¤Î¤â¤Î¤ÈÁê»÷¤·¤Æ¤¤¤ë¤È¤¹¤ëʸ¸¥¤¬¤¢¤ë. *14

Extranodal NK/T-cell lymphoma, nasal type¤¬âдݤËȯ¾É¤¹¤ë. ¹âÅÙ¿¯½±À­, CD56ÍÛÀ­, ºÙ˦¾ã³²À­ðùγ¥¿¥ó¥Ñ¥¯ÍÛÀ­, EBVÍÛÀ­¤Çͽ¸åÉÔÎɤΥê¥ó¥Ñ¼ð¤Ç¤¢¤ë.

 
 

¥¹¥Æ¡¼¥¸, ¼£ÎŤª¤è¤Óͽ¸å

âдݤò¿¯½±¤¹¤ë°­À­¥ê¥ó¥Ñ¼ð´µ¼Ô¤µ¤ó¤Î70-80%¤Ï¸Â¶ÉÀ­¼À´µ¤Ç¤¢¤ê, Ann Abor stage¤ÏI¤¢¤ë¤¤¤Ï­¶¤Ç¤¢¤ë*15*16 *17Ⱦ¿ô°Ê¾å¤Ïstage I¤Ë¤Ê¤ë.*18

âдÝDLBCL¤Ï°ìÈ̤Ëͽ¸åÉÔÎɤȹͤ¨¤é¤ì, ¼£ÎŤ¬¤¤¤¯¤é¤«¿ÊÊ⤷¤¿¸½ºß¤Ç¤â, ´µ¼Ô¤µ¤ó¤ÎÀ¸Â¸Ãæ±ûÃͤÏ6¥õ·î¤Ë²á¤®¤º, ²þÁ±¤Î;ÃϤ¬Â礭¤¤.*19

ºÆÈ¯»þ¤Ë¤ÏCNS¤Ø¤ÎȯÀ¸¤¬ºÇ¤â¿¤¯, ÂЦ¤ÎâдÝ, ¹ü, ÇÙ, ÈéÉæ, ¥ï¥ë¥À¥¤¥¨¥ëÎØ, ´Î¡, ¿Õ¡, ¤½¤Î¾¤Ø¤ÎºÆÈ¯¤â¤¢¤ë.
¥ê¥ó¥ÑÀá¤Ë¤âºÆÈ¯¤¬¤¢¤ê¤¨¤ë. *19*20*21

Diffuse large B-cell lymphoma

Case01; 80 yo male. 20ÆüÁ°¤³¤í¤è¤ê±¢Ç¹¼ðÂç¤Ëµ¤¤Å¤¯. 5Æü¸å, âдݤÎÄˤ߽и½.¡¡ÍâÆüµßµÞ³°Íè¼õ¿Ç. º¸ÀºÁã¤Ï·ÜÍñÂç¤Ë¼ðÂç. ¼£ÎŤˤè¤ë²þÁ±¤Ê¤¯¹â°ÌÀºÁãŦ½Ð½Ñ¤¬»Ü¹Ô¤µ¤ì¤ë.

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¼ðáçÆâ¤Ë²õ»à¤¬Ç§¤á¤é¤ì¤ë. °Û·¿¥ê¥ó¥Ñµå¤ÎÌ©¤ÊÁý¿£¤Ë¤è¤êÀººÙ´É¤Ï¾Ã¼º¤·¤Æ¤¤¤ë.¡¡
¼ðáçºÙ˦¤Ïcentroblastic cells. mitosis¤äapoptosis¤¬Â¿¤¤.

Diffuse large B-cell lymphoma of the lt. testis, 6.0x5.0x4.5cm in size.

 

Case02¡¡71yo male.¡¡Î¾Â¦ÀºÁã¼ðÂç¤Ëµ¤¤Å¤¯. ÀºÁã¤Ïξ¦¤È¤â¤Ë·ÚÅÙ¼ðÂ礷¹Å¤¯, °µÄˤϤʤ¤. LDH 242 U/L¤È·ÚÅÙÁý²Ã. CT, MRI¤Ç˵ÂçÆ°Ì®¥ê¥ó¥ÑÀá¤Î¼ðÂ礢¤ê.

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lt. testicular tumor

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rt. testicular tumor

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ÀººÙ´É¤Ï¿»½á¤Ë¤è¤ê°à½Ì, ¾Ã¼º. ¤Ó¤Þ¤óÀ­¤Ëcentroblasts/immunoblasts¤ÎÁý¿£¤¬Ç§¤á¤é¤ì¤ë. ¼ðáçºÙ˦´Ö¤Ë·ÚÅÙ¤ÎÀþ°Ý²½¤¬¤¢¤ë.

ξ¦À­ÀºÁã¥ê¥ó¥Ñ¼ð¤Ï, Ìȱ֥°¥í¥Ö¥ê¥ó·Úº¿°äÅÁ»ÒºÆÊÔÀ®¤Ë¤ª¤¤¤Æ°ì´Ó¤·¤¿¥¯¥í¡¼¥óÀ­¤ò¼¨¤·, ÀºÁã¤Ë¤ª¤¤¤Æ¥ê¥ó¥Ñ¼ð¤ÎÀøºßŪÇżï¤Ë¤è¤ë¤â¤Î¤Ç¤¢¤ê, ¸¶È¯À­¤Îξ¦À­ÀºÁã¥ê¥ó¥Ñ¼ð¤Ç¤Ï¤Ê¤¤¤È¿ä»¡¤µ¤ì¤Æ¤¤¤ë.*22

 

Case 03

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Áý¿£Áã¤Ç¤ÏÀººÙ´É¤Ï¾Ã¼º, °à½Ì. ¼ðáç¶­³¦Éô¤Ç¤Î¿»½á½ê¸«¤¬¤¢¤ë.

 
 

Extranodal NK/T-cell lymphoma, nasal type of the testis

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¾®¥ê¥ó¥Ñµå¤ÈÆ±ÄøÅÙ¤«1.5Çܤ¯¤é¤¤¤Î¥µ¥¤¥º, round/ convoluted nuclei¤ò¤â¤Ä¥ê¥ó¥Ñµå¤¬¥·¡¼¥È¾õÌ©¤ËÁý¿£¤¹¤ë. ³Ë¥¯¥í¥Þ¥Á¥ó¤ÏÁƤÇ, ³Ë¾®ÂΤ¬Ç§¤á¤é¤ì¤ë³Ë¤â¤¢¤ë.
DLBCL¤È¤ÏºÙ˦¤ÎÂ礭¤µ¤äºÙ˦½ê¸«¤¬°Û¤Ê¤Ã¤Æ¤¤¤ë. ÀººÙ´É¤Ø¤Î¿»½á, Àêµò½ê¸«¤¬¤¢¤ë.

 

ÌȱÖÀ÷¿§

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CD3¦ÅCD56EBVGranzyme B

Extranodal NK/T-cell lymphoma, nasal type of the testis

 
 

Case; Diffuse medium-sized B-cell lymphoma with germinal center phenotype

Nmz-case 55yo male º¸âдݤμðáî¤Ëµ¤¤Å¤­¼õ¿Ç.

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ÀººÙ´É¤Î¿¤¯¤¬¥¹¥Ú¥¢¤µ¤ì, ´Ö¼Á¤Ëlymphoid cells¤¬Ì©¤Ë¿»½á¤·¤Æ¤¤¤ë. ¤ï¤º¤«¤ÊÀººÙ´É¤Ë¿»½á¤¬Ç§¤á¤é¤ì°à½Ì¤äÇ˲õÁü¤¬Ç§¤á¤é¤ì¤ë.

 
 
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Îà±ß·Á/¿ÎÇ·Á¤Î³Ë¤ò¤â¤ÄN/CÈæÂç¤ÎºÙ˦. ÁÆÂç¤Ê¥¯¥í¥Þ¥Á¥ó¤¬Áý²Ã, ³Ë¾®ÂΤÏÉÔÌÀÎÆ. DLBCL¤Ëǧ¤á¤é¤ì¤ëcentroblasts/ immunoblasts¤ÎºÙ˦½ê¸«¤È°Û¤Ê¤ë.

LCA(CD45)+, CD20+(focal¤Ë±¢À­), CD79a+, PAX5+, CD5-, CD10+, BCL6+¤¬Â¿¤¤, MUM1-, BCL2+, cyclinD1-, MIB1 LI; very high(>80%). EBER-ISH-.

IGH; clonal band+ (BIOMED2, FR1 and FR2). MYD88L265P¤ÏAS-specific PCR¤Ç±¢À­. Molecular ¤Ë¤âcommon type¤Îtesticular DLBCL¤È¤Ï°Û¤Ê¤Ã¤Æ¤¤¤ë¤è¤¦¤Ç¤¢¤ë.


*1  Hematopathology Jaffe ES, et al.
*2  Zucca E, Conconi A, Mughal TI, et al: Patterns of outcome and prognostic factors in primary large-cell lymphoma of the testis in a survey by the International Extranodal Lymphoma Study Group. J Clin Oncol 2003; 21: 20-27
*3  Ferry J, Young R, Malignant lymphoma of the genitourinary tract. Curr Diagn Pathol 1997; 4:145-169
*4  Dalle J, et al: Testicular disease in childhood B-cell non-Hodgkin's lymphoma: the French Society of Pediatric Oncology experience. J Clin Oncol 2001; 19: 2397-2403
*5  Ferry JA, et al: Malignant lymphoma of the testis, epididymis, and spermatic cord. A clinicopathologic study of 69 cases with immunophenotypic analysis. Am J Surg Pathol 1994; 18: 376-390
*6  Pectasides D, et al: Anthracycline-based chemotherapy of primary non-Hodgkin's lymphoma of the testis: The Hellenic Cooperative Oncology Group experience. Oncology 2000; 58:286-292
*7  Cheah CY, et al. Primary testicular lymphoma. Blood 2014; 123: 486-493
*8  ¼Â¸³°å³Ø 2013ǯ9·î¹æ Vol.31 No.14
*9  Lambrechts A.C.,et al. Lymphomas with testicular localisation show a consistent BCL-2 expression without a translocation (14;18): a molecular and immunohistochemical study. Br J Cancer. 1995; 71:73-77
*10  Akhtar M, et al: Neutrophil-rich Ki-1-positive anaplastic large cell lymphoma presenting as a testicular mass. Mod Pathol 1996; 9:812-815
*11  Wilkins BS, et al.Morphological and immunohistochemical study of testicular lymphomas. Histopathology 1989; 15:147-156
*12  Crellin AM, et al. Non-Hodgkin's lymphoma of the testis. Radiother Oncol.1993 May;27(2):99-106.
*13  Kuper-Hommel MJ, et al. T(14;18)(q32;q21) involving MALT1 and IGH genes occurs in extranodal diffuse large B-cell lymphomas of the breast and testis. Mod Pathol. 2013 Mar;26(3):421-7.PMID:23018871
*14  Hyland J, et al. Molecular pathological analysis of testicular diffuse large cell lymphomas. Hum Pathol. 1998 Nov;29(11):1231-9.
*15  Al-Maghrabi J, et al: Primary low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type arising in the urinary bladder: report of 4 cases with molecular genetic analysis. Arch Pathol Lab Med 2001; 125:¡¡332-336.
*16  Kakizaki H, et al: Malignant lymphoma in the female urethra. Int J Urol 1994; 1: 281-282
*17  Lucioni M, Nicola M, Riboni R, et al: Antibiotic therapy-induced remission of bladder mucosa-associated lymphoid tissue (MALT) lymphoma carrying t(11;18)(q21;q21) apoptosis inhibitor 2-MALT1. J Clin Oncol 2013; 31: e304-e306
*18  Hatcher PA, and Wilson DD: Primary lymphoma of the male urethra. Urology 1997; 49:142-144
*19  Al-Maghrabi J, Kamel-Reid S, Jewett M, et al: Primary low-grade B-cell lymphoma of mucosa-associated lymphoid tissue type arising in the urinary bladder: report of 4 cases with molecular genetic analysis. Arch Pathol Lab Med 2001; 125: pp. 332-336.
*20  Hatcher PA, and Wilson DD: Primary lymphoma of the male urethra. Urology 1997; 49:142-144
*21  Kakizaki H, et al: Malignant lymphoma in the female urethra. Int J Urol 1994; 1: 281-282
*22  Bentley R.C., et al. Genotypic divergence precedes clinical dissemination in a case of synchronous bilateral B-cell malignant lymphoma of the testes. Hum Pathol. 1993; 24: 675-678

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