caso seminario - seap

Dra. Isabel Alvarado_Cabrero

DRA.ISABEL ALVARADODRA.ISABEL ALVARADO--CABREROCABRERO

HOSPITAL DE ONCOLOGIAHOSPITAL DE ONCOLOGIAIMSS, MEXICO IMSS, MEXICO

keme2.tijax12@gmail.comkeme2.tijax12@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

Dra. Isabel Alvarado_Cabrero

Ovarian Sarcomas

Sertoli-Leydig Cell Tumor

(Heterologous element)

Germ Cell Tumor

(GCT)

Scully RE.Tumors of the ovaryAFIP, 1998:313

Dra. Isabel Alvarado_Cabrero

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

Dra. Isabel Alvarado_Cabrero

Malignant Germ Cell Tumors

Immature Teratoma

Squamous cell caAdenocarcinomas

Malignant Melanoma

PNET

Mature Teratoma

Int J Surg Pathol 2010;18:184-192

Dra. Isabel Alvarado_Cabrero

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

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

• 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

Dra. Isabel Alvarado_Cabrero

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