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Introduction
Ewing’s sarcoma/Primitive neuroectodermal tumors (EWS/PNET)
comprise a group of rare malignant and aggressive tumors, known to
originate from migration of embryonic cells of the neural crest. They
characterstically display small round cell morphology and variable
neuroectodermal differentiation. These tumors have a predilection
for long bones, pelvis, and ribs, occuring commonly in the children
and adolescents.
1
Although extraskeletal involvement of soft tissues
either primary or metastatic is quite rare; few cases involving the
lung, pleura and lymph node have been reported.
2
Extra-mammary
metastasis to the breast is quite uncommon with prevalence ranging
from 1.7 to 6.6% and is an indicator of disseminated disease with a poor
prognosis.
3,4
Lymphoma, malignant melanoma, rhabdomyosarcoma
and lung carcinoma are common tumors that metastasize to the breast.
Sarcomas and more specifcally Ewing’s sarcoma are extremely rare
source of origin of breast metastasis with only few cases reported in
the literature till date.
4,5
Ancillary techniques are necessary for accurate diagnosis
and typing of malignant tumors of breast, due to variation in their
prognosis and ways of management. Owing to their usual superfcial
and accessible locations, fne needle aspiration cytology is usually an
important screening tool to evaluate the lesion as benign or malignant
and if malignant whether primary or metastasis. However, cytology
and histopathological biopsy along with immunohistochemical
examination forms the mainstay of diagnosis. Moreover, highlighting
the characteristic transcript t (11, 22) at molecular level is necessary
for a confrmatory diagnosis.
6
This rare case report shows the
importance of a keen cytological and histopathological examination
in early diagnosis of metastatic palpable breast lump from a primary
Ewing’s sarcoma of femur to assure prompt treatment and avoid any
unnecessary radical operation.
Case summary
A 22 year old female presented to the surgical OPD with a gradually
increasing painful lump in the right breast since the past 3 months. She
also complained of low grade fever with loss of weight and appetite
for 5-6months duration. There was no history of cough, chest pain,
any loco-regional trauma or any signifcant family history. She also
complained of gradually increasing pain in left knee since 6 months
with decreased mobility. On examination, the patient was average
built with adequate nutrition. Systemic examination was apparently
normal with presence of mild pallor. Breast examination revealed
a frm, irregular, non-tender and mobile lump of size 3cmx2cm in
upper outer quadrant of right breast without any skin, lymph node or
deep structural involvement. A doppler ultrasound was suggestive of
malignancy with BIRADS grade IVa.
Local examination of left knee joint showed an ill-defned frm
swelling and tenderness with decreased mobility but absence of any
redness or ulceration. X-ray of left knee joint showed an ill-defned
lytic lesion with mild periosteal reaction in distal femur along with
pathological fracture. Magnetic resonance imaging on both T1
and T2 weighted signals showed an ill-defned mass of increased
heterogenesity infltrating into adjacent tissue. On the basis of which a
differential of Ewing’s sarcoma and chronic osteomyelitis was given.
A fne needle aspiration cytology from both the sites of lesion was
performed which showed strikingly similar cytological features of
individually scattered as well as cohesive clusters of dual population
of cells, comprising of small round hyper chromatic cells with scant
cytoplasm and large atypical round to polygonal cells with increased
nuclear-cytoplasmic ratio, coarse granular chromatin, indistinct
nucleoli and scant cytoplasm. Occasional foci of rosette formation
and nuclear moulding were also seen Figure 1.
Based on clinic-pathological fndings a diagnosis of small blue
round cell tumor probably Ewing’s sarcoma of left distal femur with
metastasis in right breast was made. For confrmation, an incisional
biopsy from both the sites was performed which showed tumor areas
comprising of sheets of cells, with predominantly small round cells
with hyper chromatic nuclei, scant clear cytoplasm and indistinct cell
Adv Cytol Pathol. 2017;2(2):63‒66 63
© 2017 Akhtar et al. This is an open access article distributed under the terms of the Creative Commons Attribution License, which
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Ewing’s sarcoma of femur with metastasis to the
breast: a rare case report
Volume 2 Issue 2 - 2017
Kafl Akhtar, Alia Ehsan, Asim I Khan, Mubarak
Shraim
Department of Pathology, Aligarh Muslim University, India
Correspondence: Kafl Akhtar, Department of Pathology,
Jawaharlal Nehru Medical College, Aligarh Muslim University,
Aligarh (UP), India, Email drkaflakhtar@gmail.com
Received: October 14, 2016 | Published: March 22, 2017
Abstract
Ewing’s sarcoma/Peripheral primitive neuroectodermal tumors ((EWS)/PNET)
are highly malignant small round cell tumors, occurring primarily in limb bones of
children and young adults. Usual sites of metastasis are lung, pleura, other bones and
occasionally to lymph node, central nervous system and liver. Metastasis to the breast
is an extremely rare entity with only few cases reported till date. Nevertheless, it
should always be considered as a differential if there is a history of Ewing’s sarcoma
in a patient presenting with a palpable breast lump. Breast metastasis is indicative of
advanced disease with poor prognosis requiring prompt diagnosis and treatment. We
hereby present a case of a 22 year old female presenting with a palpable breast lump of
3 months duration, who had a past history of partially treated knee swelling 6 months
back. Fine needle aspiration cytology from the breast lump as well as the lower end
of femur revealed a similar morphology comprising of dimorphic population of small
round cells and larger cells, singly and in rosettes with reticulo-granular chromatin and
scant cytoplasm. Based on the immuno-histopathological examination of the excised
tissue, the diagnosis of Ewing’s sarcoma femur metastasizing to the breast was made.
Keywords: breast, cytology, ewing′s sarcoma, histopathology, metastasis
Advances in Cytology & Pathology
Case Report
Open Access