LETTER TO THE EDITOR
How to Manage Idiopathic Granulomatous Mastitis: Suggestion of an Algorithm To the Editor: Idiopathic granulomatous mastitis (IGM) is an uncommon, chronic, noninfectious inflammation of breast tissue with granuloma formation. Presentations and imaging findings usually mimic malignancies (1), which may lead to unnecessary surgical interventions and psychological insult to the patients according to fearfulness of breast cancer (2). On the other hand, considering a malignant disease as IGM may cause delay in diagnosis and treatment of breast cancer and end up to mismanagement of the cancer. Therapeutic approaches consist of both medical and surgical interventions, but there is no coalition on the best treatment, and no study with high level of evidence regarding treatment strategy was published yet. Surgical intervention may help the patients, but frequent recurrence of the attacks and considerable complications and side effects may surpass the benefit. On the other hand, radical surgical interventions may lead to exacerbation of the disease (2). Within a 4-year period, 2007–2011, we studied 20 consecutive IGM patients referred to two referral centers in Tehran, “Imam Hospital” and “Kaviani Breast Disease Institute.” The diagnosis was made histopathologically based on excisional [13], Incisional [3], and core needle [6]. Immunohistochemistry (IHC) study was performed in five specimens to rule out breast cancer. All specimens have been revised by two different pathologists to reconfirm the diagnosis. According to medical history and physical examination, appropriate imaging study was carried out for all patients. Suspicious ultra-sonographic findings for malignancy were found in six patients. Mammography was taken for 14 patients. Considering BIRADS classification, the mammograms of six patients were Address correspondence and reprint requests to: Ahmad Kaviani, Tehran University of Medical Sciences, Department of Surgery, No 3, Tavaneer Sq., Tehran, 1434888483, Iran, or e-mail: [email protected] DOI: 10.1111/tbj.12216 © 2013 Wiley Periodicals, Inc., 1075-122X/14 The Breast Journal, Volume 20 Number 1, 2014 110–112
considered as suspicious for malignancy (B5). MRI was obtained for two patients showing mastitis, microabscess, and lymphadenopathy. Pathologic diagnosis was made by observing chronic inflammation and cytologic features indicating a granulomatous process. Ziehl–Neelsen staining was performed for all tissue species to detect acid-fast bacilli and fungi, which were all negative. Culture species showed no growth after 1 and 4 weeks. None of the patients presented with Lofgren’s syndrome (3) of fever, erythema nodosum, and arthralgia in favor of sarcoidosis. Chest x-ray was obtained for all patients, which did not show hilar lymphadenopathy or any other lesions, and the pathologist was also asked to report any clue for sarcoidosis. Initial medical treatment consisted of a widespectrum antibiotic for at least 2 weeks and a nonsteroidal anti-inflammatory drug (NSAID) for at least 2 months. Systemic corticosteroids (CS) were only prescribed after 4 weeks of intractability and for early recurrent (recurrence in the first 4 weeks) or progressive disease. CS was tapered after 1 week of remission and discontinued in 2 months. All patients were followed up serially at least for 18 months. Further interventions, such as surgery (resection or drainage), were reserved for the persistent and completely intractable diseases (failure of medical treatment in 6 weeks and any sign of abscess formation). Abscess drainage was carried out for six patients. Three patients had more invasive surgical intervention, e.g., abscess wall excision, thickened area resection, and breast-conserving surgery by means of lumpectomy. In follow-up of the patients, six patients did not develop any signs and symptoms again. Severe attack, which needed surgical intervention, was observed in two patients within 9 and 12 months of follow-up. One of them showed fistula formation and was treated surgically. Excellent control was achieved in six patients (32%) and they did not show any sign and symptom
letter to the editor • 111
of recurrence during the 18 months of follow-up. Nine patients (48%) are located in “good control” category of follow-up (mild recurring attacks in
3- to 6-month interval). Two patients (10%) in fair control group (severe attack in first 3 months) and the remaining two patients (10%) who needed to use CS
Figure 1. Suggested diagnostic and therapeutic algorithm for idiopathic granulomatous mastitis (IGM).
112 • letter to the editor
were disease-resistant (without any response for 3 months). According to the review of the medical literature, there is no approved standard therapeutic plan for the IGM. While some suggest conservative medical treatment (4), others recommend surgery as soon as the diagnosis is made (5). Blind empirical antibiotics (not covering tuberculosis), corticosteroids, and surgery are the most common modalities being used for the patients, although there is no specific protocol in treatment (6,7). In another study, surgery with different kinds of resection is recommended if it would be possible to reach a good cosmetic result in breast reconstruction (8). Chronic course is not rare and it is reported from 16% to 50% in different studies (4,6,9,10); thus, long-term follow-up should be in schedule (8). Fistula formation, which can be seen as early and late, may be caused secondarily as an adverse effect of the surgery and is associated with poor outcome. Considering unpredictable surgical side effects, which can be even worse than the primary lesion, medical treatment is superior to surgical therapeutic approach at least as the first step for the treatment of the disease. The results of the current study and the acceptable outcome of the patients support the clinical application of the suggested algorithm (Fig. 1). Surgical interventions should be restricted to biopsies, draining the abscesses, and excising fistulas. We do not recommend routine surgical resection to manage IGM. In summary, while approaching a breast lesion in a female patient clinicians must always rule out the malignant diseases. As soon as the diagnosis is definitively made, the first step is to inform the patients about the chronicity of the disease and the probability of the frequent recurrence of the disease. Due to chronic nature of IGM, the treatment should begin with medical treatment and reserving surgical interventions for drainage of any kind of collections or to control the tract of probable fistula or when there was any doubt of foci of underlying malignant disease. In addition, most patients are responsive to low-dose
simple symptomatic treatments, and more potent cytotoxic and immunosuppressive drugs, such as colchicine and methotrexate, are barely needed and should be applied just for refractory cases (1). Ahmad Kaviani, MD*† Behnoud Baradaran Noveiry, MD* Khatereh Jamei, BSc† Abbas Rabbani, MD* *Department of Surgery Tehran University of Medical Sciences Tehran, Iran; † Research Department Kaviani Breast Disease Institute Tehran, Iran REFERENCES 1. Diesing D, Axt-Fliedner R, Hornung D, Weiss JM, Diedrich K, Friedrich M. Granulomatous mastitis. Arch Gynecol Obstet 2004;269:233–6. 2. Lin CH, Hsu CW, Tsao TY, Chou J. Idiopathic granulomatous mastitis associated with risperidone-induced hyperprolactinemia. Diagn Pathol 2012;7:2. 3. Neville E, Walker AN, James DG. Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients. Q J Med 1983;52:525–33. 4. Lai EC, Chan WC, Ma TK, Tang AP, Poon CS, Leong HT. The role of conservative treatment in idiopathic granulomatous mastitis. Breast J 2005;11:454–6. 5. Tse GM, Poon CS, Ramachandram K, et al. Granulomatous mastitis: a clinicopathological review of 26 cases. Pathology 2004;36:254–7. 6. Bani-Hani KE, Yaghan RJ, Matalka II, Shatnawi NJ. Idiopathic granulomatous mastitis: time to avoid unnecessary mastectomies. Breast J 2004;10:318–22. 7. DeHertogh DA, Rossof AH, Harris AA, Economou SG. Prednisone management of granulomatous mastitis. N Engl J Med 1980;303:799–800. 8. Taghizadeh R, Shelley OP, Chew BK, Weiler-Mithoff EM. Idiopathic granulomatous mastitis: surgery, treatment, and reconstruction. Breast J 2007;13:509–13. 9. Olfatbakhsh A, Beheshtian T, Djavid GE. Granulomatous mastitis, erythema nodosum, and oligoarthritis in a pregnant woman. Breast J 2008;14:588–90. 10. Azlina AF, Ariza Z, Arni T, Hisham AN. Chronic granulomatous mastitis: diagnostic and therapeutic considerations. World J Surg 2003;27:515–8.
How to Manage Idiopathic Granulomatous Mastitis: Suggestion of an Algorithm To the Editor: Idiopathic granulomatous mastitis (IGM) is an uncommon, chronic, noninfectious inflammation of breast tissue with granuloma formation. Presentations and imaging findings usually mimic malignancies (1), which may lead to unnecessary surgical interventions and psychological insult to the patients according to fearfulness of breast cancer (2). On the other hand, considering a malignant disease as IGM may cause delay in diagnosis and treatment of breast cancer and end up to mismanagement of the cancer. Therapeutic approaches consist of both medical and surgical interventions, but there is no coalition on the best treatment, and no study with high level of evidence regarding treatment strategy was published yet. Surgical intervention may help the patients, but frequent recurrence of the attacks and considerable complications and side effects may surpass the benefit. On the other hand, radical surgical interventions may lead to exacerbation of the disease (2). Within a 4-year period, 2007–2011, we studied 20 consecutive IGM patients referred to two referral centers in Tehran, “Imam Hospital” and “Kaviani Breast Disease Institute.” The diagnosis was made histopathologically based on excisional [13], Incisional [3], and core needle [6]. Immunohistochemistry (IHC) study was performed in five specimens to rule out breast cancer. All specimens have been revised by two different pathologists to reconfirm the diagnosis. According to medical history and physical examination, appropriate imaging study was carried out for all patients. Suspicious ultra-sonographic findings for malignancy were found in six patients. Mammography was taken for 14 patients. Considering BIRADS classification, the mammograms of six patients were Address correspondence and reprint requests to: Ahmad Kaviani, Tehran University of Medical Sciences, Department of Surgery, No 3, Tavaneer Sq., Tehran, 1434888483, Iran, or e-mail: [email protected] DOI: 10.1111/tbj.12216 © 2013 Wiley Periodicals, Inc., 1075-122X/14 The Breast Journal, Volume 20 Number 1, 2014 110–112
considered as suspicious for malignancy (B5). MRI was obtained for two patients showing mastitis, microabscess, and lymphadenopathy. Pathologic diagnosis was made by observing chronic inflammation and cytologic features indicating a granulomatous process. Ziehl–Neelsen staining was performed for all tissue species to detect acid-fast bacilli and fungi, which were all negative. Culture species showed no growth after 1 and 4 weeks. None of the patients presented with Lofgren’s syndrome (3) of fever, erythema nodosum, and arthralgia in favor of sarcoidosis. Chest x-ray was obtained for all patients, which did not show hilar lymphadenopathy or any other lesions, and the pathologist was also asked to report any clue for sarcoidosis. Initial medical treatment consisted of a widespectrum antibiotic for at least 2 weeks and a nonsteroidal anti-inflammatory drug (NSAID) for at least 2 months. Systemic corticosteroids (CS) were only prescribed after 4 weeks of intractability and for early recurrent (recurrence in the first 4 weeks) or progressive disease. CS was tapered after 1 week of remission and discontinued in 2 months. All patients were followed up serially at least for 18 months. Further interventions, such as surgery (resection or drainage), were reserved for the persistent and completely intractable diseases (failure of medical treatment in 6 weeks and any sign of abscess formation). Abscess drainage was carried out for six patients. Three patients had more invasive surgical intervention, e.g., abscess wall excision, thickened area resection, and breast-conserving surgery by means of lumpectomy. In follow-up of the patients, six patients did not develop any signs and symptoms again. Severe attack, which needed surgical intervention, was observed in two patients within 9 and 12 months of follow-up. One of them showed fistula formation and was treated surgically. Excellent control was achieved in six patients (32%) and they did not show any sign and symptom
letter to the editor • 111
of recurrence during the 18 months of follow-up. Nine patients (48%) are located in “good control” category of follow-up (mild recurring attacks in
3- to 6-month interval). Two patients (10%) in fair control group (severe attack in first 3 months) and the remaining two patients (10%) who needed to use CS
Figure 1. Suggested diagnostic and therapeutic algorithm for idiopathic granulomatous mastitis (IGM).
112 • letter to the editor
were disease-resistant (without any response for 3 months). According to the review of the medical literature, there is no approved standard therapeutic plan for the IGM. While some suggest conservative medical treatment (4), others recommend surgery as soon as the diagnosis is made (5). Blind empirical antibiotics (not covering tuberculosis), corticosteroids, and surgery are the most common modalities being used for the patients, although there is no specific protocol in treatment (6,7). In another study, surgery with different kinds of resection is recommended if it would be possible to reach a good cosmetic result in breast reconstruction (8). Chronic course is not rare and it is reported from 16% to 50% in different studies (4,6,9,10); thus, long-term follow-up should be in schedule (8). Fistula formation, which can be seen as early and late, may be caused secondarily as an adverse effect of the surgery and is associated with poor outcome. Considering unpredictable surgical side effects, which can be even worse than the primary lesion, medical treatment is superior to surgical therapeutic approach at least as the first step for the treatment of the disease. The results of the current study and the acceptable outcome of the patients support the clinical application of the suggested algorithm (Fig. 1). Surgical interventions should be restricted to biopsies, draining the abscesses, and excising fistulas. We do not recommend routine surgical resection to manage IGM. In summary, while approaching a breast lesion in a female patient clinicians must always rule out the malignant diseases. As soon as the diagnosis is definitively made, the first step is to inform the patients about the chronicity of the disease and the probability of the frequent recurrence of the disease. Due to chronic nature of IGM, the treatment should begin with medical treatment and reserving surgical interventions for drainage of any kind of collections or to control the tract of probable fistula or when there was any doubt of foci of underlying malignant disease. In addition, most patients are responsive to low-dose
simple symptomatic treatments, and more potent cytotoxic and immunosuppressive drugs, such as colchicine and methotrexate, are barely needed and should be applied just for refractory cases (1). Ahmad Kaviani, MD*† Behnoud Baradaran Noveiry, MD* Khatereh Jamei, BSc† Abbas Rabbani, MD* *Department of Surgery Tehran University of Medical Sciences Tehran, Iran; † Research Department Kaviani Breast Disease Institute Tehran, Iran REFERENCES 1. Diesing D, Axt-Fliedner R, Hornung D, Weiss JM, Diedrich K, Friedrich M. Granulomatous mastitis. Arch Gynecol Obstet 2004;269:233–6. 2. Lin CH, Hsu CW, Tsao TY, Chou J. Idiopathic granulomatous mastitis associated with risperidone-induced hyperprolactinemia. Diagn Pathol 2012;7:2. 3. Neville E, Walker AN, James DG. Prognostic factors predicting the outcome of sarcoidosis: an analysis of 818 patients. Q J Med 1983;52:525–33. 4. Lai EC, Chan WC, Ma TK, Tang AP, Poon CS, Leong HT. The role of conservative treatment in idiopathic granulomatous mastitis. Breast J 2005;11:454–6. 5. Tse GM, Poon CS, Ramachandram K, et al. Granulomatous mastitis: a clinicopathological review of 26 cases. Pathology 2004;36:254–7. 6. Bani-Hani KE, Yaghan RJ, Matalka II, Shatnawi NJ. Idiopathic granulomatous mastitis: time to avoid unnecessary mastectomies. Breast J 2004;10:318–22. 7. DeHertogh DA, Rossof AH, Harris AA, Economou SG. Prednisone management of granulomatous mastitis. N Engl J Med 1980;303:799–800. 8. Taghizadeh R, Shelley OP, Chew BK, Weiler-Mithoff EM. Idiopathic granulomatous mastitis: surgery, treatment, and reconstruction. Breast J 2007;13:509–13. 9. Olfatbakhsh A, Beheshtian T, Djavid GE. Granulomatous mastitis, erythema nodosum, and oligoarthritis in a pregnant woman. Breast J 2008;14:588–90. 10. Azlina AF, Ariza Z, Arni T, Hisham AN. Chronic granulomatous mastitis: diagnostic and therapeutic considerations. World J Surg 2003;27:515–8.
