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World J. Surg. 27, 515518, 2003 DOI: 10.

1007/s00268-003-6806-1

WORLD
Journal of

SURGERY
2003 by the Socie te Internationale de Chirurgie

Surgical Globetrotting Chronic Granulomatous Mastitis: Diagnostic and Therapeutic Considerations


Aziz Firzah Azlina, M.S.,1 Zakaria Ariza, M.B.B.S.,1 Talib Arni, M. Path.,2 Abdullah Noor Hisham, M.S.1
1 2

Department of Breast and Endocrine Surgery, Putrajaya Hospital, Precinct 7, 62250 Putrajaya, Malaysia Department of Pathology, Kuala Lumpur Hospital, Jalan Pahang, 50586 Kuala Lumpur, Malaysia

Abstract. To review the clinical presentation, histopathological features, and optimal treatment of chronic granulomatous mastitis, the authors conducted a retrospective study of 25 women admitted to a teaching hospital in Malaysia between January 1998 and December 2000 who met the required histologic criteria. The primary outcome measures were morbidity and recurrence of the disease. Thirteen patients presented with a breast mass clinically mimicking breast cancer, and 12 patients had breast induration and abscess formation. In addition, 8 of these patients had recurrent breast disease. Clinical and imaging diagnosis has often been difficult and inconclusive, so histopathology remains the optimal diagnostic tool. Of interest, 50% of patients experience recurrences, and long-term follow-up is therefore necessary. The authors concluded that, because chronic granulomatous mastitis is a rare benign breast condition that may be misdiagnosed as breast carcinoma, complete resection should be accomplished whenever possible. Steroid therapy may be an adjuvant for optimal treatment. Awareness among surgeons and pathologists should also be emphasized to avoid unnecessary misdiagnosis and treatment.

ing hospital; the patients were either referred from the Outpatient Department of the hospital or from peripheral government health clinics with outpatient services. The diagnosis was confirmed either by core needle biopsy or fine-needle aspiration cytology for the suspicious breast lesions and also from biopsy specimens taken from the abscess wall during drainage. Clinical data of the presentation, histopathology, and management were analyzed by review of medical records. Patients who had history of cosmetic treatment by injection of silicone, liquid paraffin, or beeswax into the breast parenchyma (olegranulomatous mastitis) were excluded from this study. Follow-up information was obtained from clinical reviews at monthly intervals (range, 2 to 11 months). The types of symptoms, severity, and duration were documented. The data collected were then studied and the various parameters were compared retrospectively. Results Complete follow-up information for all patients diagnosed with CGM with regard to the clinical presentation, histology report, and management was obtained for all 25 patients. The patients ranged in age from 21 to 47 years, with the exception of one patient who was 74 years old. The median age was 36.5 years. There were 19 Malay and 6 Indian ethnic patients in our series. The parity of these patients ranged from nil to six, with a mean parity of three. At the time of presentation, one patient was 28 weeks pregnant and five patients were lactating. None of these patients were on any medication or hormonal treatment. All patients except one presented with a history of a painful palpable lesion in the affected breast. These lesions were unilateral and occurred with no tendency to any particular quadrant of the respective breast. The size of the mass ranged from 1 to 10 cm with a mean of 5.5 cm. There was also no tendency for any side of the breast to be more involved (left 64%, right 36%). The mean duration of symptoms was 2.0 months. At the time of presentation, 12 women (52.0%) had a clinical impression of breast abscess. The other 13 women in this study (48.0%) had a clinical impression of a malignant breast lump. More than half the women in our series had already been treated with a course of antibiotics with or without

Chronic granulomatous mastitis (CGM) is a rare benign breast condition first described by Kessler and Wolloch in 1972 [1]. They reported five women with breast masses characterized by florid granulomatous mastitis, which was not associated with trauma, specific infections, or exogenous materials. To date, there are only approximately 120 reported cases in the international literature [2]. Although it is a benign entity, CGM clinically mimics breast cancer in terms of physical and radiological findings, often leading to disastrous consequences. At present, the definitive diagnosis can only be established and confirmed by histopathology. The aim of this study was to review the clinical presentation of CGM and its histopathological features, and to outline the diagnostic and therapeutic considerations in the management of this potentially recurrent nonmalignant breast disease. Patients and Methods A retrospective study included 25 women who met the required histological criteria of CGM and who were treated between January 1998 and December 2000. This study was performed in a teach-

Correspondence to: Abdullah Noor Hisham, M.S., e-mail: anhisham@ pd.jaring.my

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Table 1. Management and response of patients with initial clinical impression of breast abscess. Treatment Drainage plus antibiotics Drainage plus antibiotics with steroids Frequency (n = 12) 8/12 4/12 Response (follow-up 2 months) Nil Recurrent abscess (n = 2) Induration (n = 1)

Table 2. Management and response of patients with initial clinical impression of suspicious breast lesion. Treatment Steroid only Steroid + antibiotics Excision biopsy + antibiotics + steroids Fig. 1. Chronic granulomatous mastitis. Frequency (n = 13) 7/13 3/13 3/13 Response (follow-up 6 months) Recurrence (n = 1) Induration (n = 1) Induration (n = 1)

drainage before presenting at our clinic. Of these, 11 (41%) had received antibiotic therapy, and 4 (24%) had additional incision and drainage procedure performed. However, despite treatment, these abscesses failed to resolve. Of the 13 patients with the preliminary clinical impression of malignant breast lesion, the preoperative diagnosis was made either by fine-needle aspiration cytology (FNAC) (n = 3) or core biopsy (n = 9). Only one (33%) of the 3 patients who had FNAC achieved the diagnosis of CGM using the FNAC sample. The other two women who initially had FNAC done had a second biopsy (core biopsy), which later confirmed the diagnosis of CGM. These 2 patients eventually had wide excisions performed. The histology report from the excision biopsy or incision of the abscess wall during drainage confirmed the diagnosis in all cases. Microscopically, there were areas of necrosis and acute and chronic inflammatory cells with foamy macrophages, epithelioid cells, and multinucleated giant cells forming granulomas (Fig. 1). There were no caseations; however microabscesses were sometimes present. There were no malignant cells, fungi, or acid-fast bacilli seen. All patients had pus cultures, none of which showed any growth. In most cases, however, the cultures were taken after the patients had been started on antibiotic therapy. The response to various combinations of treatment in this series was variable. Oral prednisolone was given for 6 weeks at an initial dose of 60 mg daily in divided doses, which were tapered slowly with clinical improvement. Of the 12 patients who received steroid therapy, 3 patients had full-blown recurrent diseases and 3 others complained of non-tender induration after their first course of steroid treatment (recurrence rate 6/12 = 50.0%). Of the 2 patients with recurrent breast abscesses, one patient developed recurrence 8 months after a short 4-week course of steroid therapy. Nonetheless the recurrent disease responded well to a second course of steroid therapy. The other patient developed recurrence after concurrent treatment with antibiotics and steroids at the time when the wound had not completely healed. Both had another drainage and a second course of steroid therapy with good response. The three patients who complained of non-tender induration after a 6-week course of steroid therapy also completed a second course of steroid therapy, after which their induration subsided. In this series the complete response to the treatment of CGM varied from 6 weeks to 11 months. The steroid dosage of

prednisolone 20 mg three time daily was prescribed for 2 weeks and in a tailing dosage over 6 weeks depending on the clinical response. Patients who had recurrences were continued on low-dose prednisolone until complete clinical response were observed. In contrast, those who had secondary infection and abscess formation needed more time to heal. Tables 1 and 2 illustrate the data on management and response of patients in this series with the clinical impression of abscess and suspicious breast lesions. The response to steroid treatment was variable.

Discussion Breast cancer continues to be the most common cancer among Malaysian women. The strong negative social-cultural perception of the disease together with remote location in rural areas and inaccessibility to health care combine to account for the delayed presentation and the advanced stage of disease at presentation. At the same time, a misdiagnosis of breast carcinoma can easily be made in patients with chronic granulomatous mastitis. Chronic granulomatous mastitis is a rare benign breast disease condition characterised by a chronic necrotizing granulomatous condition. Although the origin remains unknown, it has been postulated that CGM could result from an autoimmune localized response to the retained and extravasated fat- and protein-rich secretions in the duct [3]. It is said to occur predominantly in women of childbearing age. The mean age of patients with CGM in this study was 36.5 years, which was comparable to that in previous studies. Most of these women were diagnosed within five years of their last childbirth. In addition, there appears to be no association with breast-feeding, smoking, or any hormonal treatment. Chronic granulomatous mastitis may manifest itself in two ways: first, as an abscess and second, mimicking a breast carcinoma. In this study, all patients presented with palpable breast lesions, which often involved a whole quadrant of the breast. The lesions are usually unilateral although there have been reported cases of bilateral breast involvement. There appears to be no tendency for the lesion to occur on particular side. The diagnosis of CGM remains a diagnosis of exclusion. Although it can mimic breast cancer clinically and radiologically, neither mammography (Fig. 2) nor ultrasonography is said to play a significant role in the differential diagnosis of

Azlina et al.: Chronic Granulomatous Mastitis

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Observation in the present study recommends excision biopsy whenever possible coupled with steroid therapy. In conditions complicated by abscesses, surgical drainage and antibiotic treatment must be advocated prior to starting steroid therapy. In this series, the use of steroids showed variable results and raises the possibility that perhaps the steroid therapy should be advocated at smaller doses but for a longer duration. In conclusion, chronic granulomatous mastitis is a rare benign breast condition that may be misdiagnosed as breast carcinoma. Because clinical or imaging diagnosis has often been difficult and inconclusive, histopathology remains the optimal diagnostic tool. In this series, we recommend complete resection whenever possible and steroid therapy as an adjuvant for optimal treatment. Because about 50% of patients experience recurrences, long-term follow-up is necessary. Awareness among surgeons and pathologists should also be emphasized to avoid unnecessary misdiagnosis.
Rsum. Cette tude rtrospective revoit la prsentation, les donnes histopathologiques et le traitement optimal de la mastite granulomateuse chronique. Site. Hpital universitaire, Malaisie. 25 femmes qui avaient les critres histologiques ncessaires, entre jan. 1998 et dc. 2000. Critres principaux. Morbidit et rcidive de la maladie. Treize femmes se prsentaient avec une masse au sein simulant cliniquement un cancer et 12 patientes avaient une induration du sein avec abcs. Huit patientes se prsentaient avec un cancer du sein rcidiv. Le diagnostic clinique et radiologique est souvent difficile et non concluant. Cependant, lexamen histopathologique reste loutil diagnostique optimal. Ce quil faut retenir est que 50% des patients ont une rcidiv et, par consquent, quune surveillance long terme est ncessaire. La mastite granulomateuse chronique est une maladie du sein rare qui peut simuler le cancer du sein. Nous recommandons pour un traitement optimal la rsection complte chaque fois que possible et une corticothrapie comme traitement adjuvant. Un indexe de suspicion lev parmi les chirurgiens et les anatomopathologistes est souligner pour ne pas mconnatre le diagnostic et y porter un traitement adapt. Resumen. Trabajo retrospectivo para revisar la clnica, hallazgos histopatolgicos y tratamiento ptimo de la mastitis granulomatosa crnica. Lugar. Hospital docente de Malasia. Casustica. 25 mujeres que reunan los criterios histolgicos requeridos tratadas entre enero de 1998 y diciembre del 2000. Principales resultados incluyendo la morbilidad y recidiva de la enfermedad. 13 pacientes presentaron una tumoracin mamaria que clnicamente se asemejaba a un cncer; 12 enfermas mostraron induracin mamaria y abscesos; 8 casos eran recidivas. El diagnstico tanto clnico como radiolgico fue con frecuencia difcil y no concluyente. El estudio histopatolgico constituye el mtodo idneo para el diagnstico. En el 50% de los casos se producen recidivas por lo que se precisa un largo seguimiento de estas pacientes. La mastitis granulomatosa crnica es una rara enfermedad benigna de la mama. El diagnstico diferencial ha de plantearse sobre todo con el cncer de mama. Como tratamiento ptimo recomendamos, la extirpacin a ser posible junto a un tratamiento adyuvante con esteroides. Esta afeccin ha de ser tenida muy en cuenta por los patlogos y cirujanos, para evitar errores en el diagnstico y en el tratamiento.

Fig. 2. Mammogram showing extensive microcalcification in a patient diagnosed with chronic granulomatous mastitis, which may mimic the appearance of breast cancer.

granulomatous mastitis [4]. Gray-scale ultrasonography has been reported to show findings specific for granulomatous mastitis in some cases, but all cases still require histopathologic confirmation for diagnosis [57]. In this study, the definitive diagnosis was only made through histopathological findings either from an excision biopsy or core needle biopsy. Exclusion of other known causes of granulomatous inflammations should be performed such as fungal, foreign body, or acid-fast bacilli. Although other autoimmune granulomatous breast conditions were also taken into consideration, we felt that further detailed investigations for this condition were not warranted as none of these patients complained of any symptoms or had a family history that might suggest these causes. Microscopically, CGM may appear with foamy macrophages and multinucleated giant cells forming granulomas and microabscesses (Fig. 1). The ducts may appear normal and there is no evidence of malignancy. Stains for fungi and acid-fast bacilli are negative. Each course of steroid therapy lasted for 6 weeks with an initial dose of 60 mg daily. Patients were followed up closely and the doses were tapered once the condition showed clinical improvement. Of our 12 patients who received steroids, 6 showed resolution after a single course of steroid therapy and 6 required two courses of steroids. Three of the 12 patients developed full-blown recurrent disease probably due to insufficient exposure to steroids. Nevertheless, because the recurrence developed 8 months after completion of the first course of steroid therapy, it is possible that CGM may lie dormant and, for unknown reasons, may recur months later. Thus, long-term follow-up is recommended in patients with chronic granulomatous mastitis. It remains uncertain whether long-term low-dose therapy may be offered as an additional measure to prevent recurrence.

Acknowledgment The authors thank the Director-General of Health Services, Malaysia, for his kind permission to publish this article.

References
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