Biomed Imaging Interv J 2006; 2(3):e36
© 2006 Biomedical Imaging and
Axillary lymphoma masquerading as inflammatory breast cancer
KL Taubman1, MBBS, FRACP,
MJ McKay2, FRANZCR, PhD
1Department of Nuclear Medicine, St Vincent�s Hospital,
2Divisions of Radiation Oncology and Research, Peter
MacCallum Cancer, Melbourne, Australia
Primary non-Hodgkins lymphoma (NHL) of the breast, and its extranodal spread
to the breast resulting from systemic lymphoma, are recognised albeit uncommon
conditions. However, lymphoma involving the axilla, presenting with the clinical
appearance of inflammatory breast carcinoma (IBC) without infiltration of breast
dermal lymphatics has not been reported previously.
As highlighted by the two cases presented here, this entity should be considered
in the differential diagnosis of patients presenting with clinical IBC. The
cases highlight the importance of careful histological analysis to distinguish
IBC from NHL, since management strategies and prognosis are quite different.
� 2006 Biomedical Imaging and Intervention Journal. All rights reserved.
Keywords: Inflammatory breast carcinoma; lymphoma;
Primary non-Hodgkins lymphoma (NHL) of the breast, and its extranodal spread
to the breast as a result of systemic lymphoma, are recognised albeit uncommon
conditions . Primary breast lymphoma (PBL) comprises 0.04-0.5%
of primary malignant cancers of the breast and 2% of all primary extranodal
PBL should be distinguished from the cases presented here, since axillary lymphoma
presenting with the clinical appearance of inflammatory breast carcinoma (IBC)
has not been reported previously. As highlighted by these cases, this entity
should be considered in differential diagnosis of patients afflicted with clinical
IBC. The cases highlight the importance of careful histological confirmation
in individuals presenting clinically as IBC, since management strategies and
prognosis are quite different.
Case 1, a female of 80 years, presented with
productive cough, dyspnoea, ankle oedema and weight loss. Her medical history
was otherwise largely unremarkable, and her performance status was poor ECOG
(Eastern Co-operative Oncology Group � III).
The patient had the classical clinical features of a left-sided IBC, including
redness, heat and oedema involving most of the skin of the breast, but without
a discreet accompanying breast mass. Mammography and ultrasound of the breast
did not detect a mass but the only abnormal feature was thickening of the skin
over the involved breast, consistent with dermal oedema. There was an ipsilateral
2.5cm x 2.5cm x 2cm firm, mobile, non-tender left ipsilateral axillary lymph
node mass. Chest radiology showed bilateral pleural effusions.
The initial clinical diagnosis was that of IBC with metastatic involvement
of the pleural space. Diagnostic and therapeutic pleural aspirations showed
atypical lymphocytes but no malignant cells. Multiple large-bore needle biopsies
of the skin of the left breast revealed dermal lymphatic ectasia with mild histiocytic
perivascular infiltration, but no evidence of malignancy. The size of her axillary
nodal mass increased to 7 x 5 cm over a four-day period and then spontaneously
decreased to 2 x 1 cm. Interestingly, the breast oedema decreased in severity
in parallel to the reduction in size of the nodal mass. Excision biopsy of the
axillary lymphadenopathy showed a diffused lymphocytic, well-differentiated
(B-cell) non-Hodgkin�s lymphoma (Rappaport Classification); B-cell small lymphocytic
lymphoma (WHO Classification).
Investigations at this point, including gallium and CT scanning, and bone marrow
analysis, were negative for involvement by lymphoma. The patient was commenced
on chlorambucil 10 mg daily and prednisone 60 mg daily for five days, in three-weekly
cycles. After one cycle of chemotherapy the patient still had residual small
bilateral pleural effusions, but her dyspnoea had improved considerably and
her axillary adenopathy had completely resolved, as had the inflammatory signs
in her breast.
Case 2, a 74 year-old female, presented with pain in the left axilla
and, like the first case, clinical inflammatory carcinoma of the left breast,
without a palpable mass. As for Case 1, the differential diagnosis included
cellulitis of the breast (see Discussion). Firm, irregular, non-tender lymph
nodes were palpable in the left axilla (3 x 3 x 2 cm) and in the ipsilateral
supraclavicular fossa (2 x 2 x 2 cm). The provisional clinical diagnosis, as
in the previous patient, was IBC. Bilateral mammography and ultrasound were
negative apart from apparent skin thickening. External examination was otherwise
unremarkable. Biopsy of clinically-involved skin over the left breast showed
a mild perivascular lymphocytic infiltrate, without definite tumour cells. A
left supraclavicular fossa lymph node excision biopsy was performed; whose pathological
examination showed follicular mixed NHL.
Staging investigations at this point were negative for systemic lymphoma involvement.
She was commenced on intravenous and oral chemotherapy comprising CVPP (cyclophosphamide
1,000 mg day 1, vincristine 2 mg day 1, procarbazine 150 mg orally and prednisone
60 mg per day orally for seven days each). She received four one-monthly cycles
and achieved a complete clinical remission after one cycle.
To our knowledge, these are the only two cases of lymphoma involving the axilla
which have clinically simulated IBC at presentation. The cases demonstrate the
importance of careful histopathology in distinguishing IBC with or without axillary
lymphadenopathy from lymphoma involving the axilla.
It should be noted that it is only for the last decade or so that such clinical
features of IBC have required a corroborating histopathological diagnosis. In
the cases presented here, obtaining histology of the masses in the regional
lymph node basins was essential to achieving correct diagnosis, as multiple
cutaneous biopsies were negative for malignancy. The present cases should also
be distinguished from primary breast lymphoma (PBL; Table 1 and Figure 1).
Figure 1 An hypothesis for the aetiology
of clinical features of inflammatory breast cancer in the
cases reported here.
Table 1 A comparison of the clinicopathological
features of inflammatory breast cancer (IBC), primary breast
lymphoma (PBL) and axilliary lymphoma presenting as IBC (AL).
IBC data was as reviewed by Giordano and Hortobagyi ; PBL
data was from Gholam, et al.  and Maounis, et al. ;
AL data was from the two cases presented here. U/K: unknown;
+: typically present; -: absent.
The presentation of both individuals fitted the clinical criteria for a diagnosis
of IBC, namely, signs of inflammation (redness, heat, induration) skin oedema
/peau d�orange with or without tenderness, and a cutaneous erysipeloid
edge. To fit the contemporary definition of IBC, these changes must be present
in greater than one-third of the breast, as they were in these cases; however,
a palpable mass need not necessarily be present .
The differential diagnosis for these cases included cellulitis of the breast.
Common features of breast cellulitis include a sudden onset, usually accompanied
by constitutional symptoms such as fever and chills and mild cutaneous oedema;
leukocytosis is usually present. The cases here had breast oedema but none of
the other signs of breast cellulitis.
Breast cellulitis typically begins in the pre-menopausal setting and particularly
during lactation . In contrast, the cases presented here
also lacked such features. Additionally, the size of axilliary adenopathy in
both patients, as well as the pleural effusions in Case 1, was more consistent
with a malignant aetiology. Nevertheless, the differential diagnosis of breast
cellulitis should be entertained at least until histo- or cyto- logical diagnosis
confirms the presence of malignant cells.
Histologically, IBC is characterised by lymphatic
infiltration by adenocarcinoma cells, with plugging of dermal lymphatics by
tumour emboli. The latter are largely responsible for the clinical
manifestations of IBC, secondary to lymphatic backpressure.
In IBC, the disease spreads predominantly via lymphatics, and oedematous skin,
and if biopsied, typically shows adenocarcinoma cells in the lymphatics .
It is well known that oedema of the arm and/or breast can occur after axillary
dissection and/or radiotherapy to the axilla, or in advanced IDC, due to lymphatic
Involvement of the axillary nodes by lymphoma could presumably also interfere
with lymphatic function. In the two cases of axilliary lymphoma (AL) simulating
IBC, multiple biopsies of areas of breast skin affected by peau d�orange
failed to reveal malignant cells but simply showed an inflammatory cell
infiltrate. We postulate that the oedematous, inflamed breasts in our patients
were due to lymphatic obstruction by lymphoma in the axilla, with retrograde
pressure build-up in lymphatics causing the physical signs in the breast. This
finding would be atypical for IBC. Some other features of the two cases were
different to IBC: one individual showed spontaneous regression of the axilliary
nodal mass (i.e. prior to therapy), and lymph node masses were firm rather than
hard (the latter expected in carcinomas) and both individuals had dramatic early
responses to relatively simple chemotherapy regimens.
Gholam D, Bibeau F, El Weshi A, et al. Primary breast lymphoma. Leuk Lymphoma 2003;44(7):1173-8.
Maounis N, Ellina E, Papadaki T, et al. Bilateral primary lymphoma of the breast: a case report initially diagnosed by FNAC. Diagn Cytopathol 2005;32(2):114-8.
Giordano SH, Hortobagyi GN. Inflammatory breast cancer: clinical progress and the main problems that must be addressed. Breast Cancer Res 2003;5(6):284-8.
Brewer VH, Hahn KA, Rohrbach BW, et al. Risk factor analysis for breast cellulitis complicating breast conservation therapy. Clin Infect Dis 2000;31(3):654-9.
Ronka RH, Pamilo MS, von Smitten KA, et al. Breast lymphedema after breast conserving treatment. Acta Oncol 2004;43(6):551-7.
| Received 26 April 2006; received in revised form 24 June 2006; accepted 11 July 2006
Correspondence: Division of Radiation Oncology,
Peter MacCallum Cancer Centre, Melbourne, Victoria, 3002,
Australia. Tel: 61 3 9656 1246; Fax: 61 3 9656 1424;
(Michael J. McKay).
Please cite as: Taubman KL, McKay MJ,
Axillary lymphoma masquerading as inflammatory breast cancer, Biomed Imaging Interv J 2006; 2(3):e36
This article has been viewed 10433 times.