tumori mekih tkiva dojke dr tibor tot

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Tumori mekih tkiva dojke Tibor Tot Falun, Švedska Dani Medicinske Dijaspore, Beograd 2011

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Page 1: Tumori mekih tkiva dojke dr tibor tot

Tumori mekih tkiva dojke

Tibor Tot

Falun, Švedska

Dani Medicinske Dijaspore, Beograd 2011

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Epithelial

Myoepithelial

Mesenchymal Epithelial

M i x e d

M

i

x

e

d

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Epithelial

Myoepithelial

Mesenchymal Epithelial

M i x e d

M

i

x

e

d

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Osnovne činjenice

• Bilo koji tumor mekih tkiva se može pojaviti i u dojci;

• U poredjenju sa carcinomom dojke, tumori mekih tkiva u dojci su veoma retki;

• Veċa je verovatnoċa da jedan tumor sa izgledom sarcoma je u stvari metaplastični karcinom ili loše differentivani phylloides tumor.

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Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy Keratin

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Metaplastic Carcinoma of the Breast

with Neuroectodermal Stromal Component

Tibor Tot, Juan Jose Badani De La Parra,

and Leif Bergkvist.

Pathology Research International 2011

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CK MNF 116

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Benigni tumori mekih tkiva

• Nespecifični za dojku

• ”Specifični” : Benign Stromal Spindle Cell

Tumors – BSSCTs

Tavassoli, Eusebi, Tumors of the Mammary Gland

AFIP Atlas of Tumor Pathology, Series 4, 2009

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Nespecifični benigni tumori

• Lipoma

• Adenolipoma

• Angiomyolipoma (PECOMA)

• Angiolipoma

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Lipoma

Capsule

Capsule

Capsule surrounding the circular / oval-shaped lesion

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Capsule surrounding the circular / oval-shaped lesion

Male lipoma

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Capsule surrounding the circular / oval-shaped lesion

Fibro-adeno-lipoma.

TDLUs

Fibrosis

Adipose tissue

Breast within the breast

Capsule

C

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Capsule surrounding the circular / oval-shaped lesion

Fibro-adeno-lipoma or “breast within the breast”

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Breast within the breast (fibro-adeno-lipoma): The fibroglandular tissue is radiopaque,

the adipose tissue is radiolucent.

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• FAL

Breast within the breast

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Capsule surrounding the circular / oval-shaped lesion

Fibro-adeno-lipoma

Lipoma

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Nespecifični benigni tumori

• Angioma

• BLAP: Benign LymphAngiomatous

Papules of the skin after radiotherapy

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Asymptomatic

Selected from screening

58 year

Right breast, upper lat

Fettily replaced breast

6x4mm circular lesion

Found also on ultrasound

Radiologic dg.:

Susp mucinous cancer

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Haemangioma of the breast

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Breast haemangioma:

Often asymptomatic

Incidental findings

Perilobular HA:

in 1,2% mastectomies

in 4,5% benign biopsies

in 11% autopsies

Rosen PP, Rindolfi RL.

Am J Clin Pathol 68:21-23, 1977

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Breast haemangioma:

1. Symptomatic - Cavernous

- Capillary

- Venous

2. Perilobular

3. Atypical

WHO 2002

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Mariscal A. et al.

Breast hemangioma

mimicking carcinoma.

Breast 2002 11:357-8.

Gopal SV et al.

Breast hemangioma

simulating an

inflammatory carcinoma

Breast J 2005 11:498-9

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Galindo LM. et al.

Atypical hemangioma of

the breast: a diagnostic

pitfall in breast

fine-needle aspiration. Diagn Cytopathol 2001 24:215-8.

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Honda SA, et al.

Hemangioma of the

breast with atypical

histological features.

further analysis of

histological subtypes

confirming their benign

character. Am J Surg Pathol 1992 16:553-60.

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Nespecifični benigni tumori

• Angioma

• BLAP: benign lymphangiomatous papules

of the skin after radiotherapy

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Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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Nespecifični benigni tumori

• Nurofibroma, perineuroma, Schwannoma,

Granular cell tumor

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• Case 1 diagnosed when the woman was

66 year old.

• No signs of recurrences

• As 80 she developed malignant lymphoma

and died of that disease.

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Granular cell tumor of the breast

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• 37 year old

• Palpable

• 10 mm

• Follow up 14 years, alive, no signs of

recurrence

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S-100

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S-100 NSE Cam5,2

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Benign stromal spindle cell tumors

(BSSCTs)

• With predominant myofibroblastic

differentiation

• With predominant adipocytic component

• With prominent fibroblastic elements

• (Leiomyoma / variants)

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BSSCT

SPINDLE CELLS

VIMENTIN +

CD 34+

ER;PR;AR

CLINICALLY BENIGN

ALL PUSHING BORDERS

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BSSCTs with predominant

myofibroblastic differentiation

• Myofibroblastoma

• Solitary

• Any age

• Monosomy 16q&13q

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Myofibroblastoma of the breast

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BSSCTs with predominant

adipocytic component

• Spindle cell lipoma

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Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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BSSCTs with

prominent fibroblastic elements

• Solitary fibrous tumor /

Hemangiopericytoma

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Leiomyom dojke

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Alpha smooth muscle actin

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Maligni tumori mekih tkiva

• Nespecifični za dojku

• ”Specifični” : Malignant Stromal Spindle

Cell Tumors – MSSCTs

Tavassoli, Eusebi, Tumors of the Mammary Gland

AFIP Atlas of Tumor pathology, Series 4, 2009

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Sarcomi dojke

• Veoma retki, < 1% od svih malignih

tumora

• Dr J Lamovec: 6/5382 malignih tumora u

Ljubljani za 1 godina

• Dalarna 2005-2011 oktobra, 1/1493

malignih slucajeva

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Nespecifični maligni tumori

• Liposarcom

• Osteosarcom

• Rhabdomyosarcom

• Neurogeni maligni tumori

• (Angiosarcom)

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Malignant Stromal Spindle Cell

Tumors – MSSCTs

• MSSCT with predominant fibroblastic

differentiation

• MSSCT with predominant myofibroblastic

differentiation

• (Leiomyosarcoma)

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Vim SMA Ker CD34

MSSCT fibroblastic + - - +/-

MSSCT myofibroblastic + + - +

Monophasic

sarcomatoid carcinoma

+ - + -

Myoepithelial carcinoma

+ + -/+ -

Based on Tavassoli, Eusebi AFIP 4, page 291

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The only difference resides in 25% of

axillary metastases in SC and virtually

never in sarcomas.

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Treatment of MSSCTs

• Mastectomy (recurrences rare)

• Breast conserving surgery (recurrences in

up to 2/3 of cases)

• Survival ?

• Metastases to the lungs, liver, GI tract,

adrenals, brain, bones, pleura and

retroperitoneum.

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MSSCT with predominant

fibroblastic differentiation

• Fibrosarcoma

• Malignant fibrous histiocytoma

• Vimentin only

• Diagnosis by exclusion

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Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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MSSCT Fibroblastic

Low-grade High-grade

Growth pattern Herringbone Storiform

Mitosis 2/HPV 12/HPV

Necrosis -/+ ++

Nuclear atypia little variable

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MSSCT Fibroblastic

Low-grade High-grade

Recurrences 63% 44%

Metastases none 25%

Deaths none 31%

Jones et al. Am.J.Surg.Pathol. 16: 667-674,1992

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MSSCT with predominant myofibroblastic

differentiation

• Synonym: Malignant myofibroblastoma;

myofibrosarcoma.

• Vimentin +, SMA +, Keratin -

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Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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Leiomyosarcoma of the breast

• Positive for vimentin, actin& desmin

• Nearly 50% of cases around nipple

• Circumscribed margins

• Mitotic count no same impact as uterus

• Moderately aggressive neoplasms

• Complete excision the ONLY treatment

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62 – year – old man with palpable lump

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Erroneous FNAB diagnosis: Gynecomastia

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Correct FNAB diagnosis: M y x o i d t u m o r

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M y x o i d l i p o s a r c o m a of the male breast

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Benign lipoma

Myxoid liposarcoma

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Angiosarcoma

• Definition: Malignant tumors composed of

neoplastic elements with morphological

properties of normal endothelial cells.

• Synonym: hemangiosarcoma;

hemangioblastoma; lymphangiosarcoma

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Angiosarcoma

• Primary (de novo) in breast parenchyma.

• Secondary in the skin and soft tissues of the arm following ipsilateral radical mastectomy and subsequent lymphedema (Stewart - Treves syndrome).

• Secondary in the skin and chest wall following radical mastectomy and local radiotherapy.

• Secondary in the skin or breast parenchyma or both following conservative treatment and radiotherapy.

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Angiosarcoma - grade 1

Interanastomosing channels filled with red

blood cells.

Hyperchromatic nuclei

Dissection of interlobular and intralobular

stroma

Glands entrapped

Mitoses and necrosis absent

Donnel et al. Am J Surg Pathol 5:629-642,1981

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Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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Angiosarcoma grade 3

Interastomosing vascular channels.

Solid areas (more than 50%)

Mitoses and necrosis present

Donnel et al. Am J Surg Pathol 5:629-642,1981

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Angiosarcoma grade 2

Features of grade I in 75% of tumour.

Rest solid areas.

Endothelial tufting

Mitoses numerous

Donnel et al. Am J Surg Pathol 5:629-642,1981

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TUFTING Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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Angiosarcoma - survival

5 - year 10 - year

Grade 1 91% 61%

Grade 2 68% 68%

Grade 3 31% 14%

Donnel et al. Am J Surg Pathol 5:629-642,1981

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48 year-old woman with

palpable lump in her left breast

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CD 34

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CD 34

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Pseudoangiomatous stromal hyperplasia

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First report 1986

Vuitch MI, Rosen PP, Erlandson RA. Pseudoangiomatous

hyperplasia of the mammary stroma.

Human Pathol 1986;17:185-91

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”prelymphatic” system linked to the main

lymph vessels in the breast

Hartveit F. Attenuated cells in breast stroma: the missing

lymphatic system of the breast.

Histopathology 1990;16:533-43

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Tumor with myofibroblastic differentiation

Powell CM, Cranor LM, Rosen PP. Pseudoangiomatous

stromal hyperplasia (PASH). A mammary stromal tumor

with myofibroblastic differentiation.

Am J Surg Pathol 1995; 19:270-7.

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Clinical manifestations:

1. Tumor forming (unilateral, firm, rubbery mass)

0,4 % of consecutive breast specimens Polger MR et al. Pseudoangoiomatous stromal hypeplasia: mammographic

and sonographic appearance. AJR 1996 166:349-52.

2. Incidental /microscopical

23 % of consecutive breast specimens Ibrahim RE et al. Pseudoangoiomatous hypeplasia of the mammary stroma;

some observations regarding its clinicoapathologic spectrum.

Cancer 1989 63 1154 -60

3. Massive bilateral (peau d’orange)

(1 case in Rosen’s Breast Pathology)

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Clinical manifestations:

Massive (bilateral) (peau d’orange)

- 1 case in Rosen’s Breast Pathology

- 1 case: Mansouri D et al. Pseudoangiomatous stromal hyperplaia of mammary

stroma: a case with gigantomastia. Ann Pathol 2004; 24:179-82

+ 1

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Tumor forming PASH: 1 male case

Seidman JD et al. Rapid growth of pseudoangiomatous hyperplasia

of mammary stroma in axillary gynecomastia in an immunosuppressed man.

Arch Pathol Lab Med 1993:117:736-8

PASH:

frequent incidental finding in gynecomastia

(23 – 47 %) Milanesi MF et al. Pseudoangiomatous hyperplasia of mammary stroma

associated with gynecomastia J Clin Pathol 1998:51 204-6

Badve S, Soane JP. Pseudoangiomatous stromal hyperplasia of male breast.

Histopathology 1995 26:463-6

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Male, 25 y

Bilateral gynecomasti

following testosterone

intake

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Radiologic appearance

A single case of round well-circumscribed tumor,

3.6 cm 8.2cm in 12 months

Taira N et al. Nodular pseudoangiomatous stromal hyperplasia of mammary

troma in a case showing rapid tumor growth. Breast Cancer 2005; 12:331-6

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Most of the women are premenopausal,

If postmenopausal HRT

15 –55 (36) ys.

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Puthi S et al.

Tamoxifen in the management of

pseudoangiomatous stromal hyperplasia.

Breast J 2001: 7:434-9

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CD 34

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CD 34

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CD 34

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CD 34

SMA

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Normal interlobular stroma PASH

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Normal interlobular stroma PASH

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CD 34 CD 34

Normal interlobular stroma PASH

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Tumors infiltrating PASH spaces

Damiani S, Eusebi V, Peterse JL. Malignant neoplasms

infiltrating pseudoangiomatous stromal hyperplasia of

the breast: an unrecognized pathway of tumour spread.

Histopathology 2002 41; 208-15.

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- Collagenization of the interlobular stroma

- Empty anastomosing spaces

- Myofibroblasts resembling endothel,

CD 34 +

Actin +/-, Vim +,

CK-, FVIII-

progesterone +/-

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Lui PC et al.

Fine-needle aspiration cytology of pseudo –

angiomatous stromal hyperplasia of the

breast. Diagn Cytopathol 2004 30:353-5.

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Differential diagnosis:

1. Myofibroblastoma - Fascicular PASH

2. Low-grade angiosarcoma

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PASH Myofibroblastoma (malignant)

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Differential diagnosis:

1. Myofibroblastoma - Fascicular PASH

2. Low-grade angiosarcoma

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CD31

PASH Angiosarcoma

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Hvala !