CASE PRESENTATION

I. IDENTITY

Date of hospital entry : April, 24 th, 2018

Name : Mrs. F

Age : 36 years

Gender : Female

Address : Susukan

Religion : Islam

Marital status : Married

II. ANAMNESIS

Main Complaint

Patients complained of a lump on the left breast since 2 months ago.

History of Disease

Patients come to Arjawinangun Hospital with complaints lump on the left breast since 2

months ago. Lump color resembling skin color around, not itchy, no pain, no red, well

defined, smooth texture, hard palpable, sharply bounded not soft, not interfere with

movement and activity and can be shaken. The longer, lump is felt enlarged progressively,

lump in left breast-sized like chicken egg or bigger. Complaints of fever, headache,

vomiting, nausea, and bloating are denied. A history of high blood pressure and diabetes

denied. The history of trauma and exposure to chemicals denied.

 History of Past Disease

Patient said she never had experienced the same symptoms before. The patient had no

history of surgery.

History of Family Disease

Mrs. F said, there are no family members with the same disease as patient.

III. PHYSICAL EXAMINATION

a. Present Status

General condition : Mild pain

Awareness : Compos mentis

Blood pressure : 110/80 mmHg

Pulse : 87 x/minute, regular

Respiratory : 21 x/minute

Temperature : 36,7 oC

Head

Form : Normocephalic, symmetrical

Hair : Black, no hair fall

Eye : Anemic conjunctivas (-/-), icteric scleras (-/-), light

relexes (+/+), isochore pupil right = left

Ear : Normal form, cerumen (-), thympany membrane

intact

Nose : Normal form, septum deviation (-), epitaxis(-/-)

Mouth : Normal
Neck

Enlargement of lymph nodes (-), trachea in the middle, no mass found

Thorax

Lungs – Pulmonary

Inspection : the chest is symmetrical both left and right

Palpation : fremitus vocal and tactile are symmetrical,

crepitation (-), tenderness (-), rebound tenderness (-)

Percussion : Sonor sound in both lung fields

Auscultation : Vesicular abd bronchial sound in the entire lung

field, rhonchi (-/-), wheezing (-/-)

Cor
Inspection : Ictus Cordis not visible
Palpation : Cordis Ictus palpable
Percussion : cardiac boundaries within normal limits
Auscultation : BJ I-II Reg G (-) M (-)

Abdomen

Inspection : Flat, symmetrical, mass (-)

Palpation : Tenderness (-), rebound tenderness (-)

Percussion : Tympanity sound in four quadrants

Auscultation : Intestine sound (+).

 Extremities

Upper

Muscle Tone : normal

Movement : active / active

Mass :-/-

Strenght : 5555/5555

Oedema :-/-

Lower

Muscle tone : normal

Movement : active / active

Mass :-/-

Strenght : 5555 / 5555

Oedema :-/-

Genitalia

No abnormalities

b. Localized Status

Regio : Thorax Sinistra

Inspection : Mass appears with ± 10x7x5 cm size, same color as

the surrounding skin, and there are no signs of

inflammation

Palpation : Palpable hard breast mass, moving, boundary-

clearly, not soft.

c. Laboratory Examination

Routine Haematology (April, 24 th, 2018)

Test Result Unit

Full Blood
Hemoglobin 13,2 gr/dl
Hematocrit 38,6 %
Leukocyte 7,5 10e3/μL
Trombocyte 216 10e3/μL
Erythrocyte 4,38 mm3
Erythrocyte Indexes
MCV 83,5 Fl
MCH 28,3 Pg
MCHC 34,1 g/dl
RDW 12,6 Fl
MPV 4,7 Fl
PDW 16,5 Fl
Counts (DIFF)
Eosinophil 5,9 %
Basophil 0,5 %
Segmen 53,9 %
lymphocytes 33,6 %
monocytes 6,1 %
Stab %
Coagulation
Clotting time 3 minute
Bleeding time 1 minute
Clinical chemistry
Ureum Mg/dL
Creatinine Mg/dL
Immunology
HBsAg 0,01
Anti HIV Non-reactive

X-rays (April, 24 th, 2018)

Impression:
There does not appear active pulmonary TB
There does not appear enlarged heart

Examination suggestions : lump excision

IV. DIAGNOSIS

Phylloides tumor

V. DIFFERENTIAL DIAGNOSIS

FAM

Ca mamae

VI. TREATMENT

Operative

Excision of Phylloides tumor

Medicamentosa

Ceftazidine, ketorolac, ranitidine

VII. PROGNOSIS

Ad vitam : ad bonam

Ad sanationam : ad dubia

Ad fungsionam : ad bonam
 LITERATURE REVIEW

A. Definition
Breast masses are broadly classified as benign or malignant. Common causes of a benign breast
mass include fibrocystic disease, fibroadenoma, intraductal papilloma, abscess and also
phylloideus.

Phyllodes tumors are biphasic fibroepithelial neoplasms of breast which are generally classified as
benign, borderline and malignant. Borderline tumors have great potential for local recurrence.
Cystosarcoma phyllodes derived from the Greek word, sarcoma, which means the fleshy tumor,
and phyllo, meaning leaf. These tumors display characteristics of a large, malignant sarcomas,
such as take-leaf appearance when cut, and displays the epithelium, cyst-like space when viewed
histologically. Because most of the tumor was benign, his name can be misleading. Thus, the
preferred terminology is now filodes tumor.

B. Epidemiology
There was no difference in tumor frequency filodes among patients in the United States and
patients in other countries. Tumor filodes estimated at around 1% of total breast neoplasms.
Phyllodes tumors are rare broepithelial lesions. They make up 0.3 to 0.5% of female breast tumors
and have an incidence of about 2.1 per million, the peak of which occurs in women aged 45 to 49
years. The tumor is rarely found in adolescents and the elderly. They have been described as early
as 1774, as a giant type of fibroadenoma. Majority of phyllodes tumors have been described as
benign (35% to 64%).

Due to limited data, the percentage of malignant than benign tumors filodes is not well. The
reports indicate that approximately 80-95% of tumors are benign and that filodes about 10-15%
are malignant.
The characteristics of malignanat tumor filodes are as follows:
 Recurrent malignant tumor appear more aggressive than original tumor
 Pulmonary metastases is place most often, followed by bone, heart, and liver
  Most patients with metastases died within 3 years of initial therapy
 There is no treatment fot systemic metastases occurred
 Roughly 30% of patients with malignant tumors filodes died of the disease
 Predilection for tumor seems to be no filodes
 Tumor filodes almost exclusively in women. Rare case reports have been described in men
C. Etiology
At present time, the exact etiology of phyllodes tumor and its relationship with fibroadenoma are
unclear. In study, showed that most fibroadenomas have polyclonal elements and should be
regarded as hyperplasic rather than neoplastic lesions. It has been suggested that, in a proportion
of fibroadenomas, a somatic mutation can result in a mono- clonal proliferation, histologically
indistinguishable from the polyclonal element, but with a propensity to local recurrence and
progression to a phyllodes tumor which has also been supported by clonal analysis. It has also been
postulated that stromal induction of phyllodes tumors can occur as a result of growth factors
produced by the breast epithelium. Trauma, lactation, pregnancy, and increased estrogen activity
occasionally have been implicated as factors stimulating tumor growth. e nature of these factors is
unclear but endothelin-1, a stimulator of breast fibroblast growth, may be important.

D. Pathogenesis

Unlike carcinoma breast, phyllodes tumors start outside of the ducts and lobules, in the breast’s
connective tissue, called the stroma which includes the fatty tissue and ligaments that surround the
ducts, lobules, and blood and lymph vessels in the breast. In addition to stromal cells, phyllodes
tumors can also contain cells from the ducts and lobules.

Filodes tumor is non-epithelial neoplasms of the breast are the most common, although it only
represents 1% of breast tumors. This tumor has a smooth texture, sharply bounded and usually
move freely. This tumor is relatively large tumor, with an average size of 5 cm.However, lesions
> 30 cm have been reported.
E. Classification
World Health Organization divided phyllodes tumor into benign, borderline, and malignant
categories based on the degree of stromal cellular atypia, mitotic activity per 10 high power elds,
degree of stromal overgrowth (these three are main), tumor necrosis, and margin appearance.
 Table 1

Criteria Benign Borderline Malignant

Stromal
cellularity Minimal Moderate Marked
and atypia
Stromal

overgrowth Minimal Moderate Marked

Mitoses/10
high power 0–4 5–9 ≥10
fields
Well Zone of
Tumor circumscribed microscopic Infiltrative
margins with pushing invasion around tumor margins
tumor margins tumor margins

Table 2

Histological type
Criteria
Benign Borderline Malignant
Tumor margins Pushing ↔ Infiltrative
Stroma cellularity Low Moderate High
Mitotic rate (per 10 hpf) <5 5–9 ≥10
Pleomorphism Mild Moderate Severe
F. Diagnosis
1.1 Clinical Presentation

Most of the tumor arises in women aged between 35 and 55 years (approximately 20 years later
than fibroadenoma), 3, 15, 16 more prevalent in the Latin American white and Asian populations.
Few cases have been reported in men and these have invariably been associated with the presence
of gynaecomastia. It usually presents as a rapidly growing but clinically benign breast lump. In
some patients a lesion may have been apparent for several years, with clinical presentation
precipitated by a sudden increase in size.

(i) e skin over large tumors may have dilated veins and a blue discoloration but nipple retraction
is rare.

(ii) Fixation to skin and pectoralis muscles has been reported, but ulceration is uncommon.

(iii) More commonly found in upper outer quadrant with an equal propensity to occur in either
breast.

(iv) Rarely presentation may be bilateral.

(v) e median size of phyllodes tumors is around 4 cm. 20% of tumors grow larger than 10 cm (giant
phylloides tumor). ese tumors can reach sizes up to 40 cm in diameter.

(vi) A significant proportion of patients have history of broadenoma and in a minority these have
been multiple.

(vii) Palpable axillary lymphadenopathy can be identi ed in up to 10–15% of patients but <1% had
pathological positive nodes.

1.2. Radiological Investigations

Mammography and ultra- sonography are mainstay of routine imaging of breast lumps. Wurdinger
et al show that round or lobulated shape, well-de ned margins, heterogeneous internal structure,
and nonenhancing internal septations are more common ndings in phyllodes tumors than in
fibroadenomas.
1.2.1. Ultrasonography

Lobulated shape (in some cases round or oval) well circumscribed with smooth margins, echogenic
rim, and low level homogenous internal echoes. Fluid- lled cle s in a predominantly solid mass
(highly suggestive of phyllodes tumor) with good thorough transmission and lack of microcalci
cation are seen.

1.2.2. Color Doppler Ultrasonography

Features suggesting malignant behavior are :

(i) marked hypoechogenicity

(ii) posterior acoustic shadowing

(iii) ill-de ned tumor margins

(iv) higher values of RI (resistance index)

(v) increased PI (pulsatility index)

(vi) increased Vmax (systolic peak ow velocity).

1.2.3. Mammography

(i) It shows well circumscribed oval or lobulated mass with rounded borders.

(ii) A radiolucent halo may be seen around the lesion due to compression of the surroundings.

(iii) Coarse calci cation (but malignant microcalcification is rare) may be present.

1.2.4. Magnetic Resonance Imaging (MRI)

The following features are mainly found in phyllodes tumor on MRI:

(i) round or lobulated shape and well-de ned margins

(ii) heterogeneous internal structure/non-enhancing septations

(iii) exhibits hypointense signals on T1-weighted images

(iv) exhibits hyper/isointense signals on T2-weighted images

(v) contrast enhancement pattern:

(a) benign lesion: 1. slow initial enhancement with persistent delayed phase;

(b) malignant lesion: 1. fast initial enhancement with plateau phage,

2. fast initial enhancement with wash-out phenomenon.

1.3. Pathological/Histological Assessment

As both phyllodes tumors and broadenomas belong to a spectrum of broep- ithelial lesions,
accurate cytological diagnosis of phyllodes tumors by ne needle aspiration can be difficult.
Cytologically, it is o en easier to differentiate benign from malignant phyllodes tumors than to
separate benign phyllodes tumors from fibroadenomas. In the correct clinical setting, the presence
of both epithelial and stromal elements within the cytological smear supports the diagnosis. Epithe-
lial cells may, however, be absent from specimens taken from malignant lesions. The presence of
cohesive stromal cells (phyllodes fragments), isolated mesenchymal cells, clusters of hyperplastic
duct cells, foreign body giant cells, blood vessels crossing the stromal fragments, and bipolar naked
nuclei and the absence of apocrine metaplasia are highly suggestive of a phyllodes tumor.
However, the value of FNAC in the diagnosis of phyllodes tumor remains controversial, with an
overall accuracy of about 63%. Core tissue biopsy is an attractive alternative to FNAC because of
the extra architectural information provided by histology compared with cytology. Komenaka et
al, found the sensitivity of core needle biopsy to be 99% and negative predictive value and positive
predictive value 93% and 83%, respectively, for the diagnosis.

1.3.1. Macroscopic Appearance

Macroscopically most small tumors have a uniform white consistency with a lobulated surface,
similar to that of a fibroadenoma. Large tumors on cut section o en have a red or grey “meaty”
consistency with fibrogelatinous, hemorrhagic, and necrotic areas with leaf like protrusions into
the cystic spaces.
1.3.2. Microscopic Appearance

Fine Needle Aspiration Cytology. The cytological diagnosis of phyllodes tumors is mainly
suggested by the presence of hypercellular stroma and the stromal elements on the smears being
more numerous than the epithelial ones. e cells on the smears were classi ed by Deen et al. in 1999,
and Jayaram and Sthaneshwar in 2002 , by comparison with small lymphocytes, in

(1) short, round/oval cells, two-size smaller than the size of a lymphocyte: considered to be
epithelial cells;

(2) long, spindle cells, three-size larger than the size of a lymphocyte: considered to be stromal
cells.

Many authors considered that the following aspects should also be taken into consideration in the
case of cytological diagnosis of phyllodes tumors:

(a) the presence of hypercellular stromal fragments;

(b) the cellular composition of the stromal fragments;

(c) the amount of naked nuclei on the background of the smears;

(d) the morphology of the naked nuclei (especially the bipolar ones).

Core Needle Biopsy

Fibroepithelial lesions with cellular stroma in breast core needle biopsy (CNB) specimens may
result in either fibroadenoma or phyllodes tumor at exci- sion. Assessment of stromal cellularity,
stromal cell atypia, mitoses, and relative proportion of stroma to epithelium are mainly helpful to
reach the diagnosis. Phyllodes tumors are usually differentiated histologically from broadenoma
by its increased stromal cellularity and mitotic activity. However, benign phyllodes tumor by de
nition lacks marked atypia and excess mitotic activity in its stromal component, and juvenile
fibroadenoma may also have cellular stroma, pre- senting a source of increased diagnostic di culty.
Diagnosis relies on the recognition of the exaggerated intracanalicular growth pattern in phyllodes
tumor. In addition, the stromal proliferation in juvenile fibroadenoma tends to be relatively
uniform, whereas in phyllodes tumor it is o en (though not always) more prominent in the
periductal areas. e stromal cellularity in phyllodes tumor may be heterogeneous. Con- sequently,
surgical excision is recommended for complete evaluation of the lesion.

Paddington Clinicopathological Suspicion Score

This outlines criteria to assist in the selection of patients for core biopsy, for use in conjunction
with existing local protocols. e aim of developing the score is to improve the rates of preoperative
diagnosis (see Box 1).

Box 1

G. Differential Diagnosis

Includes the following:

(i) Fibroadenoma
(ii) Adenoma
(iii) Hamartoma
(iv) lipoma,
(v) juvenile papillomatosis
(vi) carcinoma
(vii) sarcomas
(viii) metastatic tumor.

H. Management

Age is important in the management of these lesions. Under the age of 20, it should be treated
with enucleation, because the almost always beheve in a benign manner.

Aspiration cytology can suggest the diagnosis of tumor histology filodes but more precise in
the core needle biopsy is needed before planning treatment.

Haagensen reported one of the largest series, and recommend a wide local excision as the
primary approach to the treatment of benign tumor filoides. He had a local recurrence rate of
28% among 43 patients treated with local excision, with follow-up of at least 10 years.
However, only 3 of these recurrences requiring secondary mastectomy, and none of them died
of this tumor. Only 1 of 21 patients treated with mastectomy (simple or radical) had recurrence;
this a rapidly filoides sarcoma causing local and systemic metastasis. Higher recurrence rate
for benign than malignant rumor filodes have been reported in several series, reflecting a more
simple surgical approach to tumors estimated to be less serious.

It is clear that no-complete excision is the major determinant of recurrence in benign lesions
and secondary schools. Why the higher recurrence was reported from most of the series while
it is so well shown?, there are two main reasons: failure to anticipate the possibility of failure
to define tumor fildes and refinement that will assure complete excision. The former can be
found only with high level of suspicion, and a triple assessment at all masses prior to surgery.
Particularly important to avoid excision biopsy as a diagnostic procedure because it is almost
impossible strongly influence the extent of excision biopsy cavity, where it is performed as
primary procedures while the tumor remains in situ. For this reason, histoligical diagnosis to
be made by needle-core biopsy, or at least no more procedures than incisional biopsy.

Macroscopic complete excision, with the proposed limit of I cm, can be ensured by proper
technique. With normal excision technique while placing traction on the masses, easy to
 perfom dissection too close to the tumor at some point dissection. Reliable way to avoid this
is to a surgeon place the fingers of the left on the mass, and cut out the finger, with traction
only on the surrounding breast tissue.

For small lesions where the diagnosis is suggested by the triple assessment or macroscopic
appearance (soft, brown, fleshy appearance), the tumor should be excised with a 1 cm border
of normal breast tissue. If histologically benign, it is sufficient management, with excision
quadrantic (quarter-cicle) for intermediate lesions. Where the diagnosis was first identified on
histologic examination of excisional biopsy specimen, excision of scar tissue quadrantic
recommended in order ro ensure that qualified local clearance.

For large lesions and recurrent lesions, a good cleaning would involve a near-total mastectomy,
and we preferred a simple mastectomy, with reconstruction of the medium should be expected
of patients. There is some evidence of increasing incidence of breast carcinoma associated,
simultaneously or subsequently, in patients with tumor filoides and this is in additional reason
for the long-term follow up to careful examination of such patients.

Surgical therapy

In most cases of cystosarcoma phylloides, do the normal wide excision, with a circle of normal
tissue. There are no rules about the size limit. However, 2 cm limit for small tumors (<5 cm) and
a limit of 5 cm for large tumors (>5 cm) has been recommended. Lesions should not be “shelled
out”, as you might do with fibroadenoma, or without an acceptable recurrence rate increases.

 If the tumor to breast ratio is high enough to do segmental excision, total mastectomy, with or
without reconstruction, is an alternative.
 A more radical procedure is not generally justified
 Axillary lymph node dissection do only to nodes that are clinically suspected. However,
virtually all the nodes are reactive and do not contain malignant cells.
I. Complications
Like most breast surgery, postoperative complications of surgical treatment of tumors
filoides include the following:
 Infection
  Seroma formation
 Local recurrence and/ or distant

J. Prognosis

 Although cystosarcoma phylloides regarded as clinically benign tumor, the possibility of

local recurrence after excision is always there, especially with lessions showed malignant
histology. Tumor after initial treatment with wide local excision, which locally reccurent
ideally treated with total mastectomy.
 Metastatic disease was observed particularly in the lungs, mediastinum and bones
 If a tumor is benign, long-term prognosis was good following adequate local excision
 If the local recurrent tumor after excision, we then perfomed a local excision or total
mastectomy, especially curative.
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