As the prevalence of breast cancer has increased over recent years many cases with unusual presentation are emerging. This revue attempts to identify different types of primary malignancies that can affect the breast.

A five years review was undertaken at the Breast Unit of Azienda Ospedaliera “Santa Maria”, Terni (Italy). All cases of breast malignancy admitted to the Breast Unit between 2001 and 2005 were reviewed: 750 patients were diagnosed with malignant breast disease. Primary breast lymphoma (was found only in two cases accounting for an incidence of 0,26%. The pre-operative diagnosis was infiltrating carcinoma in both cases and the patients were treated with surgery and systemic chemotherapy; currently they are disease-free

Bezzi, Michela

Calzolari, Filippo

Lucchini, Roberta

Monacelli, Massimo

Ragusa, Mark

Sanguinetti, Alessandro

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G Chir Vol. 29 - n. 5 - pp. 230-232Maggio 2008230IntroductionBreast cancer is world’s most common mali-gnancy among women (1). It is known that the inci-dence of breast cancer varies in different ethnic grou-ps, higher figures having been reported in NorthAmerica with lower incidence rate from Lower Eastand Middle East.Primary Breast Lymphoma (PBL) is a rare neopla-sm that doesn’t show inter-racial differences in inci-dence. In addiction, its outcome remains unclear ascompared to other lymphomas. The PBL incidence is0.04% to 0.53% of all primary malignant tumors ofthe breast and 2.2% of extranodal lymphomas. Mostbreast lymphomas are diffuse B-cell type. The pro-gnosis in breast PBLs is related to the cell type and cli-nical stage, as in lymphomas located elsewhere. Diffe-rentiation of PBL from other breast tumors is difficultpreoperatively, especially by fine-needle aspirationbiopsy (FNAB) in which it can resemble breast carci-noma. We report two cases that were diagnosed as mali-gnant on FNAB and as breast lymphoma on microsco-pic evaluation of the incisional biopsy specimens. So-me Authors claim that PBL may not be rare but occa-sionally misdiagnosed as anaplastic carcinoma (2, 3).A high index of clinical suspicion and modern ima-ging are the key for the correct diagnosis.SUMMARY: Primary breast lymphoma: case reports and reviewof the literature.A. SANGUINETTI, M. RAGUSA, R. LUCCHINI, M. MONACELLI,  F. CALZOLARI, C. MISSO, N. AVENIAAs the prevalence of breast cancer has increased over recent yearsmany cases with unusual presentation are emerging. This revue at-tempts to identify different types of primary malignancies that can af-fect the breast.A five years review was undertaken at the Breast Unit of AziendaOspedaliera “Santa Maria”, Terni (Italy). All cases of breast mali-gnancy admitted to the Breast Unit between 2001 and 2005 were re-viewed: 750 patients were diagnosed with malignant breast disease.Primary breast lymphoma (was found only in two cases accounting foran incidence of 0,26%. The pre-operative diagnosis was infiltratingcarcinoma in both cases and the patients were treated with surgery andsystemic chemotherapy; currently they are disease- free.RIASSUNTO: Linfoma primitivo della mammella: casi clinici erevisione della letteratura.A. SANGUINETTI, M. RAGUSA, R. LUCCHINI, M. MONACELLI,  F. CALZOLARI, C. MISSO, N. AVENIANegli ultimi anni, con l’aumento della diagnosi di cancro dellamammella, sono emersi numerosi casi di neoplasie primitive con aspet-ti istologici e manifestazioni cliniche inusuali. Gli Autori analizzano la casistica di 5 anni dell’Unità di Senolo-gia del’Azienda Ospedaliera “Santa Maria” di Terni (Italia). Nel sud-detto quinquennio sono stati diagnosticati e trattati 750 cancri dellamammella; due di questi (0,26%) sono risultati linfomi primitivi del-la mammella. In entrambi i casi la diagnosi pre-operatoria è stata dicarcinoma infiltrante; le pazienti sono state trattate con terapia chi-rurgica e chemioterapia sistemica. Al momento sono libere da malat-tia.KEY WORDS: Breast cancer - Lymphoma - Diagnosis and reatment.Cancro della mammella - Linfoma - Diagnosi e terapia.Azienda Ospedaliera “Santa Maria”, Terni, ItalyArea Funzionale Omogenea Interaziendale di Endocrinochirurgia del Collo e Tessuti MolliAzienda Ospedaliera “Santa Maria della Misericordia” 1 Università degli Studi di PerugiaStruttura Complessa di Chirurgia Toracica © Copyright 2008, CIC  Edizioni Internazionali, RomaPrimary breast lymphoma: case reports and review of the literatureA. SANGUINETTI, M. RAGUSA1, R. LUCCHINI, M. MONACELLI1, F. CALZOLARI, C. MISSO, N. AVENIA231Primary breast lymphoma: case reports and review of the literatureCase reportsCase n. 1A 75 years old woman presented with a mass in the upper la-teral quadrant of the right breast in April 2003. The patient hadno past or family history.On admission, blood chemistry, including serum levels of tu-mor markers CEA and CA. 15-3, showed no abnormalities exceptanemia (Hb 8,7 g/dl). An ill-defined, firm and large tumor, mea-suring 6,8 x 7,3 cm, was recognized in the upper lateral quadrantof the breast; the tumor was fixed to the chest wall. Neither nip-ple-areola complex discharge nor ipsilateral axillary lymphoadeno-pathy was detected. An ultrasonography of the right breast revealed an hypoechoicand heterogeneous mass; results of a fine needle aspiration cyto-logy (FNAC) of the mass revealed infiltrating carcinoma.A right radical mastectomy was performed and the histologyof the mass revealed diffuse large B-cell lymphoma according toREAL classification.The patient received postoperatively six cycles of systemic che-motherapy with CHOP regimen (iv Cyclophosfamide 750 mg/m2;Doxorubicin 50 mg/m2; Vincristine 2 mg; and Prednisone 50 mgorally twice daily). Each cycle was three weeks apart.The patient showed remarkable tolerance to treatment andpresently is disease-free.Case n. 2A 49 years old woman came to our institution for a lump thatwas 2 cm in diameter and located in the upper lateral quadrant ofthe left breast. The patient had no past medical history or familyhistory.On admission the lesion was well circumscribed and firm,measuring 2,6 x 1,7 cm. Laboratory tests were normal. A mammo-gram revealed a tumor shadow without microcalcifications or spi-culations; FNAC was positive for class II cells.Lumpectomy and sentinel node biopsy were carried out andthe tumor was diagnosed as malignat lymphoma (stage III E byAnn Arbor staging), extranodal marginal zone B-cell lymphoma(low grade B-cell lymphoma of MALT type) by REAL classifica-tion.This case was classified as a low-intermediate risk disease andthe patient underwent, after surgery, six cycles of CHOP regimen(Cyclophosfamide 670 mg /m2 d.i.v day 1; ADM 45 mg/m2 d.i.vday 1; and Prednisolone 50 mg orally days 1-5).At the last control the patient was disease-free. DiscussionBreast cancer is the most common malignancyamong women in the world; hormonal, environmen-tal and genetic factors have been shown to play an im-portant role in the pathogenesis of breast malignan-cies. The incidence of breast cancer varies in the diffe-rent ethnic groups, and has the higher rate in NorthAmerica and Europe.Breast limphoma, either a manifestation of pri-mary extranodal disease or secondary involvement bysystemic disease, is a are malignancy. Primary breastlymphoma (PBL) has a reported incidence rangingfrom 0.12% to 0.53% of all breast malignancies. Se-condary breast lymphoma had reported incidence of0.07%, i.e. 17% of all malignancies metastatic to thebreast (4). Primary non-Hodgkin lymphoma of thebreast should fulfill following criteria: adequatepathological evaluation, with both mammary andlymphomatous infiltrate in closed association and ex-clusion of either systemic lymphoma or previous ex-tramammary lymphoma. Secondary lymphomas in-volving the breast are infrequent as well, however theyrepresent the largest group of metastatic tumors to thebreast (5-6). The relative frequency of primary versussecondary breast lymphomas is variably reported, the-refore the prevalence of one type versus the other isyet unclear.It is important to determine whether ML of thebreast originated in the breast or elsewhere; someAuthors, infact, claim that PBL may be a not so raredisease, mistaken at times for an anaplastic carcinoma,as occurred indeed in one of our cases on the initialbiopsy (7-9). Secondary breast involvement should al-so be considered when a breast mass appears or inflam-matory changes occur in a patient previously diagno-sed with lymphoma.Metastasis to the breast is generally uncommon,with a reported incidence of 1,3 – 6,5% of all breastmalignancies. Tumours commonly metastatizing tothe breast are lymphoma, leukaemia and malignantmelanoma.Breast involement by lymphoma usually manifestsas a painless mass incidentally detected by self exami-nation or clinical exams. The majority of cases are uni-lateral and B–cell histological lineage as in our cases.The mammogram often shows a homogeneus fainttumor shadow without either microcalcification orspiculation. Ultrasonography reveals a coarse internalecho, a hypoechoic mass with irregular border and so-metimes a lobulated mass representing a huge tumor(10). It is common opinion that there are not typicalimaging characteristics able to distinguish it fromother breast masses or neoplasms. This mandates aneedle biopsy or an excision biopsy in the primary cli-nical presentation.In any case, it is very difficult to distinguish PBLfrom breast cancer at early stage; often PBL is misdia-gnosed because neither excisional biopsy with frozensection nor FNAC is helpful in pathological differen-tiation (11, 12).Therapeutic management of PBL is controversialand is not completely established as yet. Some authorsreport that localized lesions within the breast have agood prognosis by radical surgical interventation alo-ne. However, many others report relapses 10 years af-ter surgery without adjuvant therapy; on the otherhand if the lymphoma is diagnosed to be of systemicorigin, surgery plus chemotherapy is more effective(13-15).232A. Sanguinetti et al.Prognosis is variable and depends on histologicalgrade and clinical stage. Reported outcomes for PBLin a series after 22,5 months of follow-up showedcomplete remission in 67% of patients, partial respon-se in 28% and progression of disease in 5%. Bilateraldiffuse disease has special predilection for pregnantand lactating women (16, 17). As far as outcome is concerned, some authors statethat PBL has a grim outlook, despite various treatmentoptions, while others believe that prognosis is betterthan that of a patient with disseminated lymphoma orbreast cancer, although survival has been deemed as in-versely linked to tumour size (16, 17). Patients who re-ceived chemotherapy and radiotherapy had a bettersurvival and a lower relapse rate.ConclusionIn conclusion, despite the increasing prevalence ofbreast cancer, PBL remains a rare event. A high indexof clinical suspicion, supported by modern imaging, isthe key in reaching the diagnosis. Currently, extensivehistopathology review is ongoing for tissue obtained inthe last decade to exclude the possibility of missedPBL cases.1. Abdel Hadi M. Breast cancer awareness among health profes-sionals. Ann Saudi Med 2000;20:135-136.2. Wiseman C, Liao KT. Primary lymphoma of the breast. Can-cer 1972;29:1705-1712.3. Giardini R, Piccolo C, Rilke F. Primary non-Hodgkin’slymphomas of the female breast. Cancer 1992;69:725-735.4. Jaffe ES, Harris NL, Stein H, Vardiman JW, eds. W.H.O clas-sification of tumours. Pathology and genetic of tumours of hae-matopoietic and lymphoid tissues. IARC Press. Lyon, 2001.5. Wong WW, Schild SE, Halyard MY, Schomberg PJ. Primarynon Hodgkin lymphoma of the breast: The Mayo Clinic Ex-perience. J Surg Oncol 2001;80:19 25.6. Gholam D, Bibenau F, El Weshi A, Bosq J, Ribrag V. Primarybreast lymphoma. Leukemia & Lymphoma 2003;44:1173-1178.7. Pinheiro RF, Colleoni GW, Baiocchi OC, Kerbauy FR, Duar-te LC, Bordin JO. Primary breast lymphoma: an uncommonbut curable disease. Leukemia & Lymphoma 2003;44:149-151.8. Domchek S, Hecht JL, Fleming MD, PinKus GS, CannellosGP. Lymphomas of the breast: primary and secondary involve-ment. Cancer 2002;94:6-13.9. Vignot S, Ledoussal V, Nodiot P et al. Non-Hodgkin’slymphoma of the breast: a report of 19 cases and review of theliterature. Clin Lymphoma 2005;6:37-42. 10. Pameijer FA, Beijerinck D, Hooenboom HH, Deuremberg JJ,Nortier JW. Non Hodgkin’s lymphoma of the breast causingmiliary densities on mammography. Am J Roentegnol1995;164: 609-610.11. Susnik B, Jordi Rowe J, Redlich PN, Chitambar C, ChangCC, Kampalath B. A unique collision tumour in breast, inva-sive ductal carcinoma and mucosa-associated lymphoid tissuelymphoma. Arch Pathol Lab Med 2004;128:99-101.12. Levine PH, Zamuco R, Yee HT. Role of fine-needle aspirationcytology in breast lymphoma. Diagn Cytopathol 2004;30:332-340.13. Gupta D, Shidham V, Zemba-Palko V, Keshgegian A. Primarybilateral mucosa-associated lymphoid tissue lymphoma of thebreast with atypical ductal hyperplasia and localized amyloido-sis. Arch Pathol Lab Med 2000;124:1233-1236.14. Babovic N, Jelic S, Javanovic V. Primary non-Hodgkin’slymphoma of the breast. It is possible to avoid mastectomy? JExp Clin Cancer Res 2002;19:149-1954.15. Coiffier B, Lepage E, Briere J et al. CHOP chemotherapy plusrituximab compared with CHOP alone in elderly patientswith diffuse large B-cell lymphoma. N Engl J Med 2002;346:235-242.16. Lyons JA, Myles J, Pohlman B, Macklis R, Crowe J, Crowno-ver A. Treatment of primary breast lymphoma: a review of 13cases. Am J Clin Oncol 2000;23: 334-336.17. Cabras MG, Amichetti M, Nagliati M, Orrù P, Mamusa AM,Angelucci E. Primary non-Hodgkin’s lymphoma of the breast:a report of 11 cases. Haematologica 2004; 89:1527-1528.References

Primary breast lymphoma: case reports and review of the literature

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Primary breast lymphoma: case reports and review of the literature

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