a case of granular cell tumor

CASE PRESENTATION

BY

Salma Tarek Mahmoud

Assistant Fellow at Ahmed Maher Teaching Hospital

A 22 ys old female patient complaining

of left breast mass.

Excision was done

Gross examination

•A fairly defined rubbery mass measured

10x7x7 mm.

•Cut section was homogenous grayish white

Microscopic examination

PAS staining was done

Cytokeratin

c

Diagnosis

Granular cell tumor

Granular cell tumor( GCTs)

Uncommon tumor thought to be derived from schwann cells.

Abrikossoff first described this tumour in 1926 as

‘‘granular cell myoblastoma’’, assuming it was of

myogenic origin; some even refer to the lesion as

‘‘Abrikossoffoma’’.

Considered benign, only rare reports of malignant

variants

Incidence

Occur in any age but are most common in

fourth, fifth and sixth decades of life, rare in

children.

It is about twice as common in women as in men.

Site

Arise in any organ as solitary painless nodule

Most frequently in oral cavity typically the tongue

Internal organs, particularly larynx, bronchus, stomach

and bile duct.

Clinically

Approximately 10–15% of patients have lesions at

multiple sites.

Multiple lesions may appear synchronously or over a

period of many years.

Gross picture

Poorly circumscribed nodule less than 3 cm

Cut section pale yellow-tan or gray.

Microscopically

Infiltrating compact nests or sheets

Bland looking large polygonal to spindle cells

Eosinophilic cytoplasmic granules

Nuclei are small dark monotypic

+/- vacoulization and clearing

Pseudoepitheliomatous

hyperplasia of the overlying

squamous epithelium, should

not be mistaken for squamous

cell carcinoma.

In close proximity to peripheral

nerve bundles

Special Stains and

Immunohistochemistry

S-100 protein: highlights cytoplasmic

granularity with strong cytoplasmic and

nuclear staining

Carcinoembryonic antigen (CEA): diffuse

immunoreactivity

Cytokeratin and (EMA) are negative

Myoglobin negative

(ER) and (PR) are negative

PAS positive diastease resistant cytoplasmic

granules

GCTs in breastIncidence

5%- 6% in breast.

Premenopausal women in 40’s, reported in adolescents, elderly women and men

May be more common in African-American women

Site

Superior medial quadrant (course of supra-clavicular nerve)

Clinical features

Usually solitary unilateral, rarely multiple

Painless firm mass, may be associated with skin retraction and nipple inversion

Gross

Cut surface is fairly

defined white - gray to

yellow

Less than 3 cm but

reported up to 9 cm.

Radiologically

X-ray

MammographySuggestive of malignancy due to apparent infiltration, stellatemass without calcification

Differential Diagnosis

D.D

Reactive histocytic lesion

Invasive breast carcinoma

Apocrine carcinoma

Myoblastomatoid, Lobular carcinoma histocytic variant

Alveolar soft part sarcoma

Metastatic malignant melanoma

Metastatic renal cell carcinoma

Reactive Histocytic lesions

Dispersed

histocytes with

mixed population of

inflammatory cells

Older age

Usually in outer

quadrant

large cells with

pleomorphic nuclei,

prominent nucleoli,

mitosis

Typically associated with

intra-ductal component

IHC: Positive for

cytokeratin, GCDFP-15

Apocrine carcinoma

Myoblastomatoid invasive lobular

carcinoma, histocytic variant Older age

Associated with

infiltrative

component

Loosely cohesive

tumor cells

IHC: Positive for

cytokeratin

Alveolar soft part sarcoma

• Deep soft tissue

• Pleomorphic cells

• Cells are divided into packets by thin walled vessels

• Alveolar pattern if cells discohesive

• Vascular invasion common

• IHC: Positive for Myoglobin

Metastatic malignant melanoma

Old age

History of primary

elsewhere

Nuclei usually show

nucleoli

Cytoplasmic pseudo-

inclusions

Melanin pigments

IHC: Positive HMB- 45

Metastatic renal cell tumor History of primary

elsewhere

Nests, separeted by sinasoids

Well defined cell membrane

Nuclear pleomorphism

Prominent nucleoli in high grades

Clearing of cytoplasm

IHC: positive for EMA

Take home message GCTs is an uncommon tumor occur in any age.

5%-6% incidence in breast, inner upper quadrant.

Pose a real diagnostic challenge for physicians.

Clinically and radiologically misdiagnosed as carcinoma

Preoperative diagnosis with core needle biopsy is important because treatment is with wide excision , rather than mastectomy.

Less than 1% of all GCTs, including mammary lesions, are malignant.

The prognosis for benign GCT of the breast is excellent.

Recurrence occurs in 2-8% of individuals after excision with wide margins