tumori mekih tkiva dojke dr tibor tot

Tumori mekih tkiva dojke

Tibor Tot

Falun, Švedska

Dani Medicinske Dijaspore, Beograd 2011

Epithelial

Myoepithelial

Mesenchymal Epithelial

M i x e d

M

i

x

e

d

Epithelial

Myoepithelial

Mesenchymal Epithelial

M i x e d

M

i

x

e

d

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.

Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy Keratin

Metaplastic Carcinoma of the Breast

with Neuroectodermal Stromal Component

Tibor Tot, Juan Jose Badani De La Parra,

and Leif Bergkvist.

Pathology Research International 2011

CK MNF 116

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

Nespecifični benigni tumori

• Lipoma

• Adenolipoma

• Angiomyolipoma (PECOMA)

• Angiolipoma

Lipoma

Capsule

Capsule

Capsule surrounding the circular / oval-shaped lesion

Capsule surrounding the circular / oval-shaped lesion

Male lipoma

Capsule surrounding the circular / oval-shaped lesion

Fibro-adeno-lipoma.

TDLUs

Fibrosis

Adipose tissue

Breast within the breast

Capsule

C

Capsule surrounding the circular / oval-shaped lesion

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

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

the adipose tissue is radiolucent.

• FAL

Breast within the breast

Capsule surrounding the circular / oval-shaped lesion

Fibro-adeno-lipoma

Lipoma

Nespecifični benigni tumori

• Angioma

• BLAP: Benign LymphAngiomatous

Papules of the skin after radiotherapy

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

Haemangioma of the breast

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

Breast haemangioma:

1. Symptomatic - Cavernous

- Capillary

- Venous

2. Perilobular

3. Atypical

WHO 2002

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

Galindo LM. et al.

Atypical hemangioma of

the breast: a diagnostic

pitfall in breast

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

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.

Nespecifični benigni tumori

• Angioma

• BLAP: benign lymphangiomatous papules

of the skin after radiotherapy

Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

Nespecifični benigni tumori

• Nurofibroma, perineuroma, Schwannoma,

Granular cell tumor

• 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.

Granular cell tumor of the breast

• 37 year old

• Palpable

• 10 mm

• Follow up 14 years, alive, no signs of

recurrence

S-100

S-100 NSE Cam5,2

Benign stromal spindle cell tumors

(BSSCTs)

• With predominant myofibroblastic

differentiation

• With predominant adipocytic component

• With prominent fibroblastic elements

• (Leiomyoma / variants)

BSSCT

SPINDLE CELLS

VIMENTIN +

CD 34+

ER;PR;AR

CLINICALLY BENIGN

ALL PUSHING BORDERS

BSSCTs with predominant

myofibroblastic differentiation

• Myofibroblastoma

• Solitary

• Any age

• Monosomy 16q&13q

Myofibroblastoma of the breast

BSSCTs with predominant

adipocytic component

• Spindle cell lipoma

Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

BSSCTs with

prominent fibroblastic elements

• Solitary fibrous tumor /

Hemangiopericytoma

Leiomyom dojke

Alpha smooth muscle actin

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

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

Nespecifični maligni tumori

• Liposarcom

• Osteosarcom

• Rhabdomyosarcom

• Neurogeni maligni tumori

• (Angiosarcom)

Malignant Stromal Spindle Cell

Tumors – MSSCTs

• MSSCT with predominant fibroblastic

differentiation

• MSSCT with predominant myofibroblastic

differentiation

• (Leiomyosarcoma)

Vim SMA Ker CD34

MSSCT fibroblastic + - - +/-

MSSCT myofibroblastic + + - +

Monophasic

sarcomatoid carcinoma

+ - + -

Myoepithelial carcinoma

+ + -/+ -

Based on Tavassoli, Eusebi AFIP 4, page 291

The only difference resides in 25% of

axillary metastases in SC and virtually

never in sarcomas.

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.

MSSCT with predominant

fibroblastic differentiation

• Fibrosarcoma

• Malignant fibrous histiocytoma

• Vimentin only

• Diagnosis by exclusion

Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

MSSCT Fibroblastic

Low-grade High-grade

Growth pattern Herringbone Storiform

Mitosis 2/HPV 12/HPV

Necrosis -/+ ++

Nuclear atypia little variable

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

MSSCT with predominant myofibroblastic

differentiation

• Synonym: Malignant myofibroblastoma;

myofibrosarcoma.

• Vimentin +, SMA +, Keratin -

Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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

62 – year – old man with palpable lump

Erroneous FNAB diagnosis: Gynecomastia

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

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

Benign lipoma

Myxoid liposarcoma

Angiosarcoma

• Definition: Malignant tumors composed of

neoplastic elements with morphological

properties of normal endothelial cells.

• Synonym: hemangiosarcoma;

hemangioblastoma; lymphangiosarcoma

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.

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

Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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

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

TUFTING Case courtesy of

Professor Vincenzo Eusebi,

Bologna, Italy

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

48 year-old woman with

palpable lump in her left breast

CD 34

CD 34

Pseudoangiomatous stromal hyperplasia

First report 1986

Vuitch MI, Rosen PP, Erlandson RA. Pseudoangiomatous

hyperplasia of the mammary stroma.

Human Pathol 1986;17:185-91

”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

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.

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)

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

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

Male, 25 y

Bilateral gynecomasti

following testosterone

intake

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

Most of the women are premenopausal,

If postmenopausal HRT

15 –55 (36) ys.

Puthi S et al.

Tamoxifen in the management of

pseudoangiomatous stromal hyperplasia.

Breast J 2001: 7:434-9

CD 34

CD 34

CD 34

CD 34

SMA

Normal interlobular stroma PASH

Normal interlobular stroma PASH

CD 34 CD 34

Normal interlobular stroma PASH

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.

- Collagenization of the interlobular stroma

- Empty anastomosing spaces

- Myofibroblasts resembling endothel,

CD 34 +

Actin +/-, Vim +,

CK-, FVIII-

progesterone +/-

Lui PC et al.

Fine-needle aspiration cytology of pseudo –

angiomatous stromal hyperplasia of the

breast. Diagn Cytopathol 2004 30:353-5.

Differential diagnosis:

1. Myofibroblastoma - Fascicular PASH

2. Low-grade angiosarcoma

PASH Myofibroblastoma (malignant)

Differential diagnosis:

1. Myofibroblastoma - Fascicular PASH

2. Low-grade angiosarcoma

CD31

PASH Angiosarcoma

Hvala !