Caso Seminario - SEAP
Transcript of Caso Seminario - SEAP
Dra. Isabel Alvarado_Cabrero
DRA.ISABEL ALVARADODRA.ISABEL ALVARADO--CABREROCABRERO
HOSPITAL DE ONCOLOGIAHOSPITAL DE ONCOLOGIAIMSS, MEXICO IMSS, MEXICO
[email protected]@gmail.com
Caso Seminario
Dra. Isabel Alvarado_Cabrero
Clinical History
32 year old patient
Lower abdominal
and pelvic mass
Preoperativ
eUltrasound
Cystoscopy
RectoscopyNegative
CA-125 --118U/ml
α-fetoprotein – 5ng/ml
HCG – 7 U/ml
SERUM LEVELS
Dra. Isabel Alvarado_Cabrero
Clinical History
Total hysterectomy + BSO
Tumor debulking
Omentectomy
Metastases in the omentum
Left Ovarian Mass
17x14 cm
Abdominal Laparotomy
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Histologic Findings
(main component)
HyperchromaticSpindle cells
Mild to moderatepleomorphism
Fascicular
“Herringbone Growth pattern”
Absence expressionof any markers but
Vimentin
Dra. Isabel Alvarado_Cabrero
Histologic Findings
(second component)
Cells
Arranged in sheets
TrabeculaeLarge rounded tumor cells
Fibrous bands + lymphocytes
CD117 +
PLAP +OCT4 +
CD30 -
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Sarcomas of the Female Reproductive
Tract
• Pure sarcomas are rare
– Most commonly carcinosarcomas
– Most common site uterine corpus
(4% of all malignancies)
• Ovarian sarcomas comprise <1% of ovarian
malignancies
• Carcinosarcoma most commonly
reported ovarian
sarcoma
Semin Surg Oncol 1994;10:369-73
Dra. Isabel Alvarado_Cabrero
Fibrosarcoma
LeiomyosarcomaAngiosarcoma
Malignant Schwannoma
Osteosarcoma
Overgrowth of one
component
MMMT/AdenosarcomaOvarian Stroma
Ovarian Sarcomas
Ann Diagn Pathol 2003;7:300-305
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Ovarian Sarcomas
Sertoli-Leydig Cell Tumor
(Heterologous element)
Germ Cell Tumor
(GCT)
Scully RE.Tumors of the ovaryAFIP, 1998:313
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Dra. Isabel Alvarado_Cabrero
Germ Cell Tumors with
Sarcomatous Component
2 Retroperitoneal
46 Patients
3 Ovarian
18 Testicular
23 Mediastinal
Am J Surg Pathol 2007;31:1356-1362
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
GCT with Sarcomatous Component
• Age: 17-74 yrs (m:27)
• 43 patients were male
• 81% with advanced tumors
– Stages II/III
• 40 patients with follow-up available
– 80% DOD
• Comparison
– Group of GCTSC vs GCT/NS,
higher mortality (p≤0.001)
Am J Surg Pathol 2007;31:1356-1362
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Am J Surg Pathol 2007;31:1356-1362
• Mature or Inmature teratoma, pure or combined
was seen in >90% of the cases
• Most frequent SC: Embryonal RMS
• >90% cases: sarcoma present in the primary
tumor
• ≤10% SC present, only in the metastases
GCT with Sarcomatous Component
Am J Surg Pathol 2009;33:1173-1178
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Malignant Germ Cell Tumors
Immature Teratoma
Squamous cell caAdenocarcinomas
Malignant Melanoma
PNET
Mature Teratoma
Int J Surg Pathol 2010;18:184-192
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Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Germ Cell Tumors with
Sarcomatous Component
Theories
Primitive Germ
Cells
DedifferentationPhenomenon
Malignant Transformation
within Teratoma
Am J Surg Pathol 1998;12:75-82
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Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
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Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
• Michael et al:
– “Mesenchyme-like” component in YST:
Myxoid or collagenous stroma or skeletal
muscle and cartilage
– Mesenchyme-like areas: maybe site of
origin of the sarcomas
GCT with Sarcomatous Component
Theories
Arch Pathol Lab Med 1989;113:1115-1119
Dra. Isabel Alvarado_Cabrero
• Morphologic criteria used for the diagnosis:
– SC: 10% to >50% of the tumor
– Expansile growth pattern
– Stromal overgrowth with cytologic atypia
– Mitoses
– Infiltration of surrounding structures
GCT with Sarcomatous Component
Cancer 1984;54:1824-33
Dra. Isabel Alvarado_Cabrero
Dra. Isabel Alvarado_Cabrero
GCT with Sarcomatous Component
• Cytogenetic studies have provided some insight
into the origin:
• Motzer et al:
– 12 cases of GCT
– Presence of:
• Isochromosome 12p in 10 cases
• Deletion of chromosome 12p in 1 case
J Urol 1998;159:133-38APMIS 2007;115:1296-1301
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Dra. Isabel Alvarado_Cabrero
• Cytogenetic features of the fibrosarcoma
– Gain in chromosome 12
• Genes located in chromosome 12 (SMS, CDK4):
– Found amplified in different kinds of sarcomas
GCT with Sarcomatous Component
Gynecologic Oncology 2002;84:150-154
Dra. Isabel Alvarado_Cabrero
Ovarian Germ Cell Tumors with
Sarcomatous Component
Age (yr)
GCT Sarcoma Type FIGO stage
No. Cases
33 T Angiosarcoma III/IV 6
32 T Fibrosarcoma II 2
41 T Mitotically Active
Celular Fibroma
I 1
23 D + T Rhabdomyosarcoma III 1
13 YST Fibrosarcoma I 1
25 MT/IT Rhabdomyosarcoma I 1
29 MT/EC Leiomyosarcoma II 1
25 D/MT/IT Rhabdomyosarcoma III 1
Dra. Isabel Alvarado_Cabrero
Tx and Sampling GCT with
Sarcomatous Transformation
• Prognosis of GCT depends on:
– Site and clinical stage…
– However, emergence of SC portends a
worse prognosis
• SC may behave as an independent tumor that
can metastasize as a unique element
• Highly resistant to the standard combination
chemotherapy commonly employed for the
treatment of GCT
Am J Surg Pathol 2000;24:257-73
Dra. Isabel Alvarado_Cabrero
Follow-up, one year later
Dra. Isabel Alvarado_Cabrero
Thank you