Management of differentiated teratoma: the Growing Teratoma Syndrome GTS Roberto Salvioni M'D' Urolo

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Management of differentiated teratoma the
Growing Teratoma Syndrome (GTS)Roberto Salvioni
M.D.Urologic Unit

International Symposium on Testicular Cancer
Munich, 15-17 May 2008
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• Not always an enlargement of metastatic mass
  during or after main course of chemotherapy
  administered for germ cell tumor (GCT) means
  disease progression or relapse
• it can be the
• Growing Teratoma Syndrome (GTS)

Logothetis et al. 1982
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Growing Teratoma SyndromeWhen does it occur?

• The Growing Teratoma Syndrome is a rare clinical
  event that occurs in 1,2 to 7,6 as a result of
  chemotherapy for the treatment of germ cell
  tumor.

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Growing Teratoma SyndromePathogenesis

• Malignant cell differentiation into Mature
  Teratoma
• or
• Selective chemotherapy-induced destruction of
  components different from Mature Teratoma

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Growing Teratoma SyndromeWhere it occurs ?

• GTS can be detected with CT scan, during or
  after main course of systemic chemotherapy, in
  any sites of previous metastatic lesions from GCT
  or in new sites
• Retroperitoneum
• Lung
• Other (liver, other lymph-nodes, bone)

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Growing Teratoma SyndromeHow to identify it ?

• The diagnosis of GTS needs the following
  three criteria
• Normalized, if previously elevated, serum tumor
  markers (STM AFP/HCG/LDH)
• Clinical and/or radiologic enlargement of
  metastatic masses during or after chemotherapy
  for GCT
• Pure Mature Teratoma without malignant cells on
  pathological examination

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Growing Teratoma SyndromeRadiologic features

• CT scan in GTS may show transformation of
  metastatic lesions
• Increase density of mass lesion
• Well defined margin of the lesions
• BUT
• There is not a particular radiologic
  characteristic that can help in identifying
  malignant from benign deposits

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Growing Teratoma SyndromePredicting factors

• Presence of Mature Teratoma in the primary
• Increase of size on serial imaging during
  chemotherapy
• Normalization of previously elevated STM
• Presence of Mature Teratoma or an incomplete
  resection of post-chemotherapy residual masses
  are predictive factors for a late recurrence
  containing Mature Teratoma

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Growing Teratoma SyndromeResidual Mature
Teratoma following primary seminoma
In case of metastatic seminoma, a residual mass
containing Mature Teratoma is possible
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Growing Teratoma SyndromeWhats the problem ?

• The presence of residual and growing mass can
  cause
• Compression on nearby anatomical structures and
  pain syndrome due to mass effect
• Dysfunction of other nearby organs
• Operative morbidity and complexity
• Malignant trasformation (into NSGCT, sarcoma,
  squamous cell carcinoma, adenocarcioma,
  carcinoid, neuroectodermal tumor/PNET)

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Growing Teratoma SyndromeWhat to do and why?

• Surgery is the treatment of choice of any post
  chemotherapy NSGCT residual mass (Mature Teratoma
  is a chemo-insensitive disease)
• Surgery confirms diagnosis of GTS and can
  demonstrate pathological absence of other
  malignant elements.
• Surgery is almost always curative when the
  residual mass has been completely excised

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Growing Teratoma SyndromeConclusions

• Early detection is associated with a high rate
  of complete resection ? close CT scan-based
  surveillance of pts presenting high risk of GTS
• Local recurrences are very rare after radical
  surgery (lt 5)
• Incomplete surgery can lead to a high recurrence
  rate (gt 80)
• Complete surgery remains the optimal treatment
  with a low risk of subsequent disease progression

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CLINICAL CASES

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CLINICAL CASE 1

• 30 years-old man M.N.
• May 2007 Right Orchidectomy (STM AFP 36 UI/ml
  HCG 3.8 mUI/ml LDH 250 U/L)
• Histology of the primary ECAMTYST, no VI
• Staging at onset (elsewhere) Clinical Stage I
  with normalised markers
• Clinicians decided for adjuvant chemo from May
  2007 June 2007 PEB x 2 Cycles
• First visit at INT Milan
• February 2008 chest and abdominal CT scans
  abdominal mass gt 3 cm with normal markers (review
  of initial CT scans of May 07 CS IIA disease!)
• 27/03/2008 RPLND ? Mature Teratoma radically
  excised

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Before PEB
After PEB
Before PEB
After PEB
Before PEB
After PEB
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CLINICAL CASE 2

• 35 Years-Old man RF
• December 1996 Right orchidectomy
• Histology ECA ? (not re-assessed)
• Initial staging Stage IV (Retroperitoneal,
  Liver Brain mets)
• STM AFP 350 UI/ml HCG 35.000 mUI/ml LDH 1500
  U/L (poor prognosis according to IGCCCG
  classification)
• From January 1997 to June 1997 PEB x 6 cycles
  WB Radiotherapy
• STM normalized after third cycle of PEB
• September 1998 First restaging residual
  retroperitoneal mass (interaortocaval mass 4.8
  cm ) with normal markers
• First visit at INT, Milan
• February 2008 progressed abdominal mass
  (interaortocaval
• mass 6 cm ) with normal markers
• March 2008 RPLND ? Mature Teratoma radically
  excised

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1996 before PEB
1997after PEB
2003 after PEB
1996 before PEB
2003 after PEB
1997 after PEB
1996 before PEB
1997 after PEB
2003 after PEB
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2008 after PEB
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Acknowledgements
Surgical Dpt Urologic Unit Nicola Nicolai Luigi
Piva Davide Biasoni Silvia Stagni Tullio
Torelli Angelo Milani
Medical Oncology Dpt Andrea Necchi Massimo Di
Nicola
Pathology Dpt Maurizio Colecchia
Radiotherapy Dpt Silvia Tana
Fondazione IRCCS Istituto Nazionale dei Tumori of
Milan