MYSTERY.html
Mystery of bilateral breast masses
Nausheen Khan, FC Rad
Zaeem I Ebrahim, FC Rad
Irma van de Werke, FRCR
Department of Radiology, Kalafong Hospital and University of Pretoria
Corresponding author: Z Ebrahim (zaeem@iafrica.com)
Introduction
Leiomyosarcoma (LMS) is an uncommon malignant
tumour of smooth muscle origin. It arises in the gastro-intestinal
tract, retroperitoneum, urinary bladder, uterus and soft tissue.
Peritoneal leiomyosarcomatosis (PL) is defined as a peritoneal
dissemination of a primary sarcoma. We present a case of
leiomyosarcomatosis with widespread dissemination including involvement
of both breasts.
Case report
A 38-year-old woman, retrovirus negative, presented
at the outpatient department of Kalafong Hospital, with a 3-month
history of weight loss and enlarging bilateral breast masses. On
examination there were numerous large, hard, nodular fixed masses in
both breasts and axillae. The preliminary diagnosis at this stage was
lymphoma, bilateral breast carcinoma, or unknown primary malignancy
with metastasis to the axillae and breasts. On further examination,
masses were felt in the anterior abdominal wall and in the back and
thigh. Several soft tissue core biopsies were done of the axillary
masses. A cervical smear showed no malignant cells.
Computed tomography (CT) of the chest and abdomen
demonstrated a large complex mass arising from the uterus with both
cystic and solid components (Fig. 1). Another mass lesion was noted in
the right kidney with extension into the inferior vena cava with
complete occlusion (Fig. 2). There were several soft-tissue enhancing
masses in both breasts and axillae (Fig. 3); a mass in the anterior
abdominal wall (Fig. 4); and a mass in the pericardium (Fig. 5) and the
posterior compartment of the left thigh (Fig. 6). All the masses had
similar soft-tissue features. The diagnosis on CT was disseminated
leiomyosarcomatosis. Histological examination of the tissue submitted
showed several cores of tumour comprising pleomorphic spindle-shaped
cells. Tumour giant cells were also noted and several mitoses were
present including atypical mitosis. There were areas of myxoid
degeneration. Immunohistochemical staining of the tissue was diffusely
and strongly positive for actin, negative for desmin, and negative for
cluster of differentiation (CD) of 117. The histological and
immunohistochemical features were most in keeping with the diagnosis of
a leiomyosarcoma. Further evaluation of the uterine mass was
recommended and discussed with the patient and her prognosis explained.
She refused further intervention. She was sent home with the clinical
diagnosis of disseminated leiomyosarcomatosis.
Discussion
Leiomyosarcoma is a relatively uncommon malignant mesenchymal tumour that exhibits smooth muscle differentiation.1
,
2
It is a tumour of adult life seen more commonly in women than men, and
generally has a poor prognosis with a high percentage of recurrence.
Leiomyosarcomatosis is defined as dissemination of leimyosarcoma from a
primary in the gastro-intestinal tract to the peritoneum or omentum; or
haematogenous spread to distant sites including the head and neck, skin
and soft tissue.2 Diffuse
peritoneal and omental seeding are well-known forms of dissemination of
metastatic carcinoma either via direct invasion through the serosa, or
haematogenous or embolic spread.2
,
3 Breast metastasis from extramammary malignancy is rare, constituting 2% of breast tumours.4
Conforming to Virchow’s concept, the breast has resistance to
metastasis from extra-mammary malignancy because the breast has large
areas of fibrous tissue, with a relatively poor blood supply.4
,
5
Breast metastasis with multiple lesions from uterine leiomyosarcoma and
especially with bilateral involvement as seen in our case, is less
common, however. Primary leiomyosarcoma of the breast is rarer;4
,
6 it is usually low-grade and should be distinguished from leiomyoma.6 The average interval for occurrence of non-breast tumour metastasis to the breast is approximately 2 years.5
,
6
Our patient presented primarily with metastatic breast masses rather
than with a primary tumour in the uterus. In 85% of patients,
metastatic leiomyosarcoma presents as a solitary breast lump and only
15% as bilateral or multiple lumps as in our case. Leiomyomas and
leiomyosarcomas arise from the spindle cells of the muscular coat of
the gastro-intestinal tract and less commonly from the muscularis
mucosa.7 CT examination in
leiomyosarcomatosis usually demonstrates multiple dumbbell or oval
tumours of soft-tissue density that enhance homogenously on intravenous
contrast administration. Tumours more than 5 cm may show central
hypodensity owing to necrosis. Calcifications are uncommon.7
Conclusion
Metastases to the breasts from primary sites are
unusual and bilateral involvement even rarer. So far there have only
been a few cases reported of leiomyosarcoma to the breast. These
metastases to the breast are seen on an average interval of
approximately 2 years after the detection of the primary site. In our
case, the patient presented primarily with metastasis. The diagnosis
becomes a formidable issue when the breast metastasis represents the
first event in the patient’s work-up. The presence of metastatic
tumour in the breast carries a poor prognosis, and radical procedures
are unnecessary and can be avoided.
1. Akata D, Aralasmak A, Ozmen MN, et al. US and CT findings of multicentric leiomyosarcomatosis. Eur Radiol1999;9:711-714.
1. Akata D, Aralasmak A, Ozmen MN, et al. US and CT findings of multicentric leiomyosarcomatosis. Eur Radiol1999;9:711-714.
2. Choi BNI, Lee WJ, Chi JG, Han JK. CT manifestations of peritoneal leiomyosarcomatosis. AJR 1990;10(155): 799-801.
2. Choi BNI, Lee WJ, Chi JG, Han JK. CT manifestations of peritoneal leiomyosarcomatosis. AJR 1990;10(155): 799-801.
3. Rha SU, Ha HK, Kim AY, et al. Peritoneal leiomyosarcomatosis
originating from gastrointestinal leiomyosarcomas: CT features.
Radiology 2003;227:385-390.
3. Rha SU, Ha HK, Kim AY, et al. Peritoneal leiomyosarcomatosis
originating from gastrointestinal leiomyosarcomas: CT features.
Radiology 2003;227:385-390.
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metastases from primary leiomyosarcoma. Aust NZ J Surg 1997;67:71-72.
4. Tulasi NR, Kurian S, Mathew G, Viswanathan FR, Roul RK. Breast
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retroperitoneal leiomyosarcoma metastasizing to the breast. Diagn
Cytopathol 2007;35(8):508-511.
6. Omeroglu G, Erssahin C, Potkul RK, Booth CN. FNA diagnosis of
retroperitoneal leiomyosarcoma metastasizing to the breast. Diagn
Cytopathol 2007;35(8):508-511.
7. Korman U, Ersavasti G, Kurugoglu S,Uygun NT, Akman C. Radiologic
evaluation of gastrointestinal leiomyosarcomatosis. Eur Radiol
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evaluation of gastrointestinal leiomyosarcomatosis. Eur Radiol
1997;7:1332-1334.
Fig.
1. Sagittal post-contrast CT through the pelvis demonstrates a large
hetrogenous mass with areas of low densities representing necrosis
(asterisks) arising from the anterior superior aspect of the uterus.
Arrow points to the uterus.
Fig. 2. Axial CT post-contrast through the upper
abdomen. There is a large mass in the right kidney (open arrow) with
complete occlusion of the IVC (black arrow). There is also a
soft-tissue mass in the right paraspinal mass (asterisk).
Fig. 3. Axial CT post-contrast through the chest at
the level of the breasts. There are bilateral breast masses and a mass
in the right axilla.
Fig. 4. Solitary mass in the anterior abdominal wall. Note a markedly dilated IVC (asterisk).
Fig. 5. Mass in the pericardium (arrow) and left axilla.
Fig. 6. Similar soft tissue mass in the posterior compartment of the left thigh.