Case Report, J Womens Health Issues Care Vol: 2 Issue: 6
A Rare Diagnosis of Malignant Fibrous Histiocytoma of the Breast
| Nicole Stamatopoulos*, Penelope De Lacavalerie and Davendra Segara | |
| Department of Breast and Endocrine Surgery, University of New South Wales, Australia | |
| Corresponding author : Nicole Stamatopoulos Department of Breast and Endocrine Surgery, University of New South Wales, 14/30-34 Raymond St, Bankstown NSW 2200, Australia Tel: 0414 821-821 E-mail: nic96@hotmail.com |
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| Received: May 05, 2013 Accepted: October 26, 2013 Published: November 01, 20132 | |
| Citation: Stamatopoulos N, De Lacavalerie P, Segara D (2013) A Rare Diagnosis of Malignant Fibrous Histiocytoma of the Breast. J Womens Health, Issues Care 2:6. doi:10.4172/2325-9795.1000123 |
Abstract
A Rare Diagnosis of Malignant Fibrous Histiocytoma of the Breast
Primary breast osteosarcoma is an extremely rare diagnosis and accounts for 1% of all breast cancers. Malignant fibrous histiocytomas are an even more rare cancer, especially de novo. Surgery is the first line treatment. However, further medical treatment remains to be investigated. This is a case of a 59 year old female who presented to a breast surgeon with a self-diagnosed right breast lump.
Keywords: Breast; Osteosarcoma; Australia
Keywords |
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| Breast; Osteosarcoma; Australia | |
Introduction |
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| Every so often in medicine, a typical work up of a patient turns into an extremely rare diagnosis. The diagnosis is so rare that there is very little evidence in the literature to assist in treatment of the patient. At times, even the most experienced in their medical field are a loss as to know what to do for the patient. This is the case with a patient who presented with a breast lump and had pathology that had the experts unsure as to what to do. | |
| A 59 year old female presented to her General Practitioner (GP) with a self-diagnosed right sided breast lump that she had neglected to have investigated for several months for personal reasons. Past medical history was unremarkable, was not on any regular medication and had no allergies to any. Her mother had breast cancer at 89 years old and died with the disease. Her GP referred her for triple assessment including a core biopsy. She was referred to a breast surgeon for further evaluation. | |
| On examination, the patient had a 3×4 cm mobile lump in the upper inner quadrant of the right breast, 6 cm from the nipple, not fixed to superficial or deep tissues with no associated lymphadenopathy. The contralateral breast was normal. Triple assessment included a two view Mammogram (Figure 1) and Ultrasound (Figure 2) [1]. Initial core biopsy identified sheets of pleomorphic ovoid and polygonal cells, with mild to moderate nuclear atypia with adjacent areas of necrosis. The lesion could not be definitively characterised. Repeat core biopsy identified overall changes consistent with a giant cell tumour of low malignant potential. | |
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Figure 1: Mammogram- magnified craniocaudal (CC) and medial lateral oblique (MLO) views showed a rounded opacity 4.2×3.2 cm in the upper inner quadrant of the right breast with a well-defined margin, uniform in attenuation and no calcification. |
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Figure 2: Ultrasound at core biopsy showed a mainly solid breast lesion with macro lobulations, well defined margins, cystic heterogeneous areas and minor increased doppler blood flow signal. This lesion measured 3.9×2.7×2.8 cm lesion and was located at 7.2 cm from the nipple. |
| After discussion with the patient, a partial mastectomy and sentinel node biopsy was performed. | |
| Histopathology of the surgical specimen showed Grade III extraskeletal osteosarcoma (osteoblastic variant) with giant cells and no mesenchymal differentiation, in 35 mm in maximal macroscopic dimension, the lateral and superficial margins of excision were close to the margin with a wider lateral excision tumour free (Figure 3). | |
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Figure 3: Histopathology of wide local excision a) low power b) high power. |
| In view of the histopathology results, further staging was performed including a bone scan and a CT with IV contrast of the chest, abdomen and pelvis. This was performed one month following the partial mastectomy. Staging failed to reveal any metastatic disease. | |
| The patient’s presentation, operative and pathological findings were discussed at a Multidisciplinary meeting with specialists from surgery, pathology, radiology and medical oncology. The consensus amongst these professionals was for the patient to undergo a complete right breast mastectomy. The patient followed the recommendation of total mastectomy. The findings from that operation confirmed residual tumour of the same type as in the partial mastectomy. The margins were clear. | |
| In view of the pathological findings and the rarity of this tumour, medical and radiation oncology specialists agreed that this tumour should be treated as a soft tissue sarcoma. | |
| Prior to the treatment, a specialised sarcoma unit tertiary centre gave the overall opinion that in the absence of osteoid within the specimen, the appearance was in keeping with a grade 3 undifferentiated pleomorphic sarcoma (FNCLCC), or malignant fibrous histiocytoma. | |
| A second opinion from an alternative tertiary centre was sought. They agreed that the tumour was a high grade undifferentiated pleomorphic sarcoma with frequent osteoclastic type giant cells (giant cell malignant fibrous histiocytoma). A follow up chest CT was performed in view of the above diagnosis. This time it showed evidence of metastatic disease. The patient underwent chemotherapy including two cycles of adriamycin and cyclophosphamide, one cycle of adriamycin alone, followed by 6 cycles of carboplatin. | |
Discussion |
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| This is the first documented case of malignant fibrous histiocytoma of the breast in Australia. Malignant histiocytomas are very rare, particularly in isolation. It is rare that malignant fibrous histiocytomas grow de novo. They usually develop after radiation treatment [2]. Only 30 cases have been reported in the literature [3]. | |
| The initial diagnosis is primary osteosarcoma of the breast is also rare. There are approximately 63 cases of breast osteosarcoma that have been published in the English language literature since 1996 [4]. It is an extremely rare cancer, accounting for less than 1% of all primary breast malignancies [4-6]. The frequency is difficult to ascertain because of its rarity [6] and many reports include sarcomatous overgrowth of phyllodes tumours [4]. Breast osteosarcoma is not confined geographically or to any ethnicity. While rare, it has a poor prognosis, is usually identified late and frequently recurs. It would be beneficial to consider whether a treatment strategy is necessary to treat this subtype of breast cancer. | |
| When examined, these tumours are mobile, have an irregular outline and do not have any associated axillary lymphadenopathy [7]. Mammography often shows a well-circumscribed dense lesion with coarse calcifications [4] and an irregular or lobulated mass [4]. They can look deceptively benign [6]. In this patient, it is partially true. There were lobulations in the histopathology specimen, but no definite calcifications. | |
| From a surgical perspective, mastectomy improves the disease free survival period compared with wide local excision alone [4,6]. Wide local excision is linked to a higher rate of recurrence. | |
| Axillary dissection has no role in these cancers as they are commonly spread haematogenously [4,6] metastasizing to the lung [4,6,8]. This is the case in the current patient, given that repeat CT showed metastatic deposits in the lungs. | |
| Histologically, these tumours are fibroblastic, osteoclastic (giant cell rich) or osteoblastic in subtypes [4,6]. | |
| The role of chemotherapy or radiotherapy in these cancers is unclear [7]. Several studies have advocated for adjuvant chemotherapy. However, these patients have had previous radiotherapy, trauma or local recurrent metastases. In one study, there were four out of five patients who did receive adjuvant chemotherapy following surgery who remained disease free [9]. | |
| In terms of survival, larger tumours tend to have a poorer survival outcome [4,6,7]. This appears to be the most significant predictor of survival outcome [6]. | |
| Long term prognosis is generally poor, due to the extremely aggressive biological behaviour with a high rate haematogenous metastatic spread and low axillary lymph node involvement presence [10]. Fibroblastic osteosarcoma types have a significantly better 5 year survival rate (67%) than those with the osteoblastic or osteoclastic subtype (31%) [5]. This was also found in other studies that report a 38% five year survival rate given the biologically aggressive nature of mammary osteosarcoma (38%) [11]. | |
| The patient responded well to chemotherapy treatment and has remained stable. Two and a half years following treatment, she has had no tumour progression and is a survivor. | |
References |
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