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


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


Clinical History

Total hysterectomy + BSO

Tumor debulking

Omentectomy

Metastases in the omentum

Left Ovarian Mass

17x14 cm

Abdominal Laparotomy


Histologic Findings

(main component)

HyperchromaticSpindle cells

Mild to moderatepleomorphism

Fascicular

“Herringbone Growth pattern”

Absence expressionof any markers but

Vimentin


Histologic Findings

(second component)

Cells

Arranged in sheets

TrabeculaeLarge rounded tumor cells

Fibrous bands + lymphocytes

CD117 +

PLAP +OCT4 +

CD30 -


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


Fibrosarcoma

LeiomyosarcomaAngiosarcoma

Malignant Schwannoma

Osteosarcoma

Overgrowth of one

component

MMMT/AdenosarcomaOvarian Stroma

Ovarian Sarcomas

Ann Diagn Pathol 2003;7:300-305


Ovarian Sarcomas

Sertoli-Leydig Cell Tumor

(Heterologous element)

Germ Cell Tumor

(GCT)

Scully RE.Tumors of the ovaryAFIP, 1998:313


Germ Cell Tumors with

Sarcomatous Component

2 Retroperitoneal

46 Patients

3 Ovarian

18 Testicular

23 Mediastinal

Am J Surg Pathol 2007;31:1356-1362


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


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


Am J Surg Pathol 2009;33:1173-1178


Malignant Germ Cell Tumors

Immature Teratoma

Squamous cell caAdenocarcinomas

Malignant Melanoma

PNET

Mature Teratoma

Int J Surg Pathol 2010;18:184-192


Germ Cell Tumors with


Theories

Primitive Germ

Cells

DedifferentationPhenomenon

Malignant Transformation

within Teratoma

Am J Surg Pathol 1998;12:75-82


• 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


Theories

Arch Pathol Lab Med 1989;113:1115-1119


• 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


Cancer 1984;54:1824-33



• 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


• Cytogenetic features of the fibrosarcoma

– Gain in chromosome 12

• Genes located in chromosome 12 (SMS, CDK4):

– Found amplified in different kinds of sarcomas


Gynecologic Oncology 2002;84:150-154


Ovarian Germ Cell Tumors with


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


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


Follow-up, one year later


Thank you

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