CC BY
9
9. Jacobs B.S., Boden-Albala B., Lin I F., Sacco R.L. Stroke in the young in the northern Manhattan stroke study // Stroke. -2002. - Dec. 33(12). - P. 2789-93.
10. Kitamura A., Iida M., Makagava Y., kiyama M et al. Characteristics and outcome of stroke patients discharger from hospital in an urban area of Japan // Nippon. Koshu. Eisei. Zasshi. - 1998. - Vol. 45(8). - P. 740-48.
11. Ramirez Lassepas M. Stroke in the aging of the brain and the arteries // Geriatrics. - 1998. - 53 Suppl 1. - P. 544-48.
12. Reith W. Spontaneous intracerebral hemorrhage: the clinical neuroradiologycal view // Radiology. - 1999. - Oct. 39(10). - P. 828-37.
13. Tatu L., Moulin T., El Mohamad R. et al. Primary intracerebral hemorrhage in the Besanson stroke registry. Initial clinical and CT finding, early course and 30-day outcome in 350 patients // Eur. Neurol. - 2000. - 43(3). P. 209-14.
14. Wolfe C.D., Tilling K.., Beech R., Rudd A.C. Variations in case fatality and dependency from stroke in western and central Europe. The European BIOMED Study of Stroke are Group // Stroke. - 1999. - Feb. 30(2). - P. 350-6.
A CASE OF STROMAL SARCOMA OF THE MALE BREAST
Peshev Zhivko Vladimirov
Assistant Professor at the Department of General and Clinical Pathology and Forensic Medicine, Medical University, Plovdiv,
Bulgaria
Danev Vladimir Hristov
Assistant Professor at the Department of General and Clinical Pathology and Forensic Medicine, Medical University, Plovdiv,
Bulgaria
Novakov Ivan Petkov
Associate Professor at the Clinic of Special Surgery, St George University Hospital, Plovdiv, Bulgaria
ABSTRACT
The primary breast sarcomas are very rare tumors - up to 1% from all neoplasms in this area. They are even more rarely found in men. We report the case of a 58-year old man who had two surgeries on sarcoma of the mammary gland within a period of three years. The tumor is histologically presented with diffuse proliferation of elongated cells with moderate atypia, single mitoses and focal necrosis. The conducted immunohistochemical study proved the occurrence of stromal sarcoma. This is a tumor of high malignancy where
except surgical removal, it is also recommended to conduct radiation therapy. Hormonal treatment and polychemotherapy show no effect.
Keywords:breast sarcoma, stromal sarcoma, male breast.
Introduction
Sarcomas of the mammary gland are very rare. In a big series of Mayo Clinic their frequency is less than 1% of all breast tumors and about 5% of all mesenchyme tumors [9]. They spread out of the mesenchymal stroma of the breast. They are mainly found in the fourth and fifth decade of age. They are exceptionally rare with men. Only a few cases are reported in the world literature.[11,12,13]
Case Presentation
The patient is a 58-year old man who had a surgery on tumor of the right breast at the beginning of 2010. Mastectomy and lymph dissection were performed but no other treatment was administered. In 2011, he was operated again for tumor recurrence and refused the following polychemotherapy. One year later he entered the Clinic of General Surgery at Sveti Georgi University Hospital and presented himself with two
tumor formations arising from the operative cicatrix of the right breast, without pain, with oval shape and diameter of 6 cm and 3 cm, with firm consistency, well distinguished, capsulated, with grayish-whitish colour, the cut section appeared like fish meat. CAT scan of the chest cavity and abdomen did not display the presence of increased lymph nodes or organ metastases. All the paraclinical tests of the patients are within the normal. Extirpation of both tumors and plastic reconstruction of the operative cicatrix were performed. After a 7-day stay, the patient was discharged in good condition and referred for radiation therapy.
The histological examination found diffuse proliferation of elongated cells with moderate cell atypia and single mitoses, against the background of swollen stroma with light myxomatous changes. (Fig. 1; Fig. 2)
Fig. 1
НЕ - х 10
Fig. 2 НЕ - х 10
In the bigger tumor were also found sections of necrosis. (Fig. 3) The immunohistochemical test for smooth muscle actin, desmin, CD117, pan-cytokeratin, CK7 and CK20, CD 10, CD 34, ER, PR and S-100 was negative. Ki 67 is 10%. Only Vimentin
was diffusely positive, with clear and strong expression in the tumor cells. (Fig. 4) The histochemical tests with Sudan III and van Gieson were also negative.
The conclusion is that it is a stromal sarcoma of the mammary glaii' ¥
< Щ
ft ЛЛ . _ 'jw i
Fig. 3 HE - x 10
Discussion
The breast sarcomas are malignant neoplasms growing out of the mesenchyme tissue and which represent part of the wide range of all connective tissue tumors. The mammary gland is a rare place for a primary sarcoma to occur and therefore its histological variants and clinical course have not been well studied.
The stromal sarcomas were first defined by Berg et al. in 1962. He excluded the malignant phyllodes tumors, lymphomas and angiosarcomas from this group, describing 25 cases of homogenous tumors with fibrosis, myxoid and fatty tissue. [8] The tumors were named stromal sarcomas, considering the normal variants of the mammary stroma. Taking this into consideration, for more than 20 years, all mesenchyme sarcomas of the breast were described as stromal sarcomas.
Later, a number of articles on the connective tissue tumors of the mammary gland began to include the fibroepithelial lesions, real connective tissue tumors without epithelial component, etc.
The lack of accurate definition, the rarity of the tumors and the different languages of the articles leads to total confusion of the terminology. To make it even more confusing, some authors begin to include in this group even carcinomas with pseudosarcomatous changes in the stroma.[2,3,8]
In the last issue of the WHO Classification of Breast Tumors of 2013, the term stromal sarcoma is only used in the description of the so called periductal stromal tumors. It also points out that if the morphological features of sarcoma and the cell origin
Fig. 4 Vimentin - x 40
can be proven, then the terms liposarcoma, leiomyosarcoma, fibrosarcoma, etc. should be used.[13] It is accepted that this classification holds more details of information, which helps for a more accurate prognosis and adequate therapy.
The most often described sarcomas of the mammary gland are fibrosarcomas and liposarcomas, whereas other sarcomatous lesions such as leiomyosarcomas are rather rare.[12] In spite of this, many authors claim that in cases with pure mesenchymal phenotype, the definition stromal sarcoma remains suitable. Morphologically it is classified as invasive, purely spindle cell tumor, only positive for vimentin. This tumor does not contain an epithelial component, one where the histological and immunohistological study cannot determine the origin of the cellular elements which build it and therefore represents a subspecies of the mammary sarcomas and has its place in the mammary gland tumors classification^ 4,9,12]
Clinical Course and Prognosis
In the literature is reported that local recurrence is observed in about half of the cases where sectoral resection of the breast has been performed and in less than 30% of the cases where total mastectomy has been performed.[13] It is suitable, despite the rare metastasis into the lymph nodes, to also make lymph dissection. There is no accurate data for the effect of the following treatment applied - radiation therapy and chemotherapy. The sarcomas of the mammary gland have no hormonal receptors and therefore the hormonal treatment is not indicated. It is considered that the
prognostic factors and survival rate of the patients is comparable to the one of sarcomas of different localization.[1,14]
Conclusion
This case meets the criteria for mesenchymal origin without epithelial component, with only Vimentin positive expression in the cells, which allows us to diagnose it as stromal sarcoma of the breast - a tumor of high malignancy, which represents an unusual variant of soft tissue sarcoma.
The uniqueness of this case is related to finding the sarcoma in a man. Despite the lack of metastases, low mitotic activity and weakly presented polymorphism, in this case we see a third-time tumor recurrence.
Literature:
1. Adem C, C Reynolds, JN Ingle and AG Nascimento: Primary breast sarcoma: clinicopathologic series from the Mayo Clinic and review of the literature. British Journal of Cancer. 2004, 91, 237 - 241
2. Abeysekara AM, HP Siriwardana, KF Abbas, P Tanner, AA Ojo.: An unusually large myofibroblastoma in a male breast: a case report. J Med Case Reports. 2008, 14, 2, 157.
3. Altintoprak F, K. Serin et all.: Undifferentiated Sarcoma Of The Breast. The journal of breast health. 2007, 3, (3), 169-172
4. Anuradha C. K. Rao, V. Geetha.: Periductal stromal sarcoma of breast with lipoblast-like stroma Indian Journal of Pathology and Microbiology. 2008, 51, 2, 252 - 254
5. Ashraf F. Hefny, O. M.. Bashir, J. Sandhya, J Frank, Fikri M. Abu-Zidan: Stromal Sarcoma of the Breast. A Case Report Asian Journal Of Surgery. 2004, 27, 4, 339-42
6. Bhattacharjee PK, S. Ghosh, T. Choudhury, SK. Mitra, S. Banerjee.: Stromal sarcoma of breast: a case report. J Indian Med Assoc., 2000, 98(4),189-90.
7. Charles D. Callery, PP Rosen, DW. Kinne.: Sarcoma of the Breast. A Study of 32 Patients with Reappraisal of Classification and Therapy. Ann Surg. 1985, 201(4):527- 32
8. Chawla S, SV Deo, NK Shukla, AK Rathi, M Prakash.: Stromal sarcoma of the breast: a case report. Indian J Pathol Microbiol. 1998, 41(3), 355-6.
9. Christensen L, T. Schi0dt, M. Blichert-Toft, J.P. Hansen, O.H. Hansen.: Sarcomas of the breast: a clinico-pathological study of 67 patients with long term follow-up. Eur J Surg Oncol. 1988, 14(3), 241-7
10. Hefny AF, MO Bashir, S Joshi, FJ Branicki, FM Abu-Zidan.: Stromal sarcoma of the breast: a case report. Asian J Surg. 2004, 27(4), 339-41.
11. Komaki K, T Morimoto, Sonoo H, Inui K, Harada K, Inoue K, Inaba H.: Stromal sarcoma of the male breast. Gan No Rinsho. 1984, 30, 2, 167-9.
12. Rao AC, V. Geetha, A. Khurana: Periductal stromal sarcoma of breast with lipoblast-like cells: a case report with review of literature. Indian J PatholMicrobiol. 2008, 51(2),252-4
13. Tavassoli FA, P Devilee. editors WHO Classification of tumours 2003, Pathology and Genetics Tumours of the Breast and Female Genital Organs. Lyon, IARC Press, 2003, 239-260.
14. Zelek L., A. Llombart-Cussac, P. Terrier, X. Pivot, J.M. Guinebretiere, C. Le Pechoux, T. Tursz, F. Rochard, M. Spielmann, and A. Le Cesne: Prognostic Factors in Primary Breast Sarcomas: A Series of Patients With Long-Term Follow-Up. Journal of Clinical Oncology, 2003, 21, 13, 2583-2588
