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Breast Mondor's disease: Diagnosis and management of six new cases of this underestimated pathology Vittorio Pasta, Valerio D'Orazi, Daniela Sottile, Luca Del Vecchio, Andrea Panunzi and Paolo Urciuoli Phlebology published online 26 September 2014 DOI: 10.1177/0268355514553494 The online version of this article can be found at: http://phl.sagepub.com/content/early/2014/09/26/0268355514553494

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Phlebology OnlineFirst, published on September 26, 2014 as doi:10.1177/0268355514553494

Original Article

Breast Mondor’s disease: Diagnosis and management of six new cases of this underestimated pathology

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Vittorio Pasta, Valerio D’Orazi, Daniela Sottile, Luca Del Vecchio, Andrea Panunzi and Paolo Urciuoli

Abstract Mondor’s disease is an unusual and little-known pathology of the breast, characterized by superficial thrombophlebitis. The causes are still unresolved. Most of the patients do not fall under case studies of the scientific literature, given the reported incidence rate between 0.5% and 0.8%. The Mondor’s disease patients are not always properly identified, and they are frequently treated as outpatients, even considering the benign course of the disease which often spontaneously resolves without any medical therapy. We report here six new cases of Mondor’s disease, two of them were likely due to a trauma and were easily resolved with the use of non-steroidal anti-inflammatory drugs; the third one was apparently due to the stretching of the mammary veins in a patient with gigantomastia; the fourth one was subsequent to hormonal stimulation for in vivo fertilization and following gestation; and the last two cases (one was a man) were diagnosed after undergoing surgery for breast carcinoma.

Keywords Mondor’s disease, breast, thrombophlebitis, non-steroidal anti-inflammatory drugs

Introduction Mondor’s disease (MD) is an uncommon condition of the breast affecting more frequently women. It is a benign, self-limiting disorder that was originally described as a superficial thrombophlebitis mainly not only of the lateral thoracic vein, a tributary vein of the axillary one, but also of the superior epigastric and thoracoepigastric veins.1 The clinical appearance of MD is of a subcutaneous, firm, not painful but tender to palpation cord, linear or serpiginous, associated or not with redness, itching, edema and retraction of the overlying skin and characterized by sclerosing thrombophlebitis of the subcutaneous veins of the chest wall.2 Although the MD may be associated with numerous pathological conditions such as accidental trauma, muscle strains, bacterial and viral infections, gigantomastia and/or drug treatments,3–6 to date no specific etiopathogenic factors have been identified. In all, 50–60% of MD cases are considered idiopathic without apparent cause and the remaining 40–50% seems to be secondary to known causes.2–7 The disease is sometimes reported in association with breast cancer with an incidence ranging from 4% to 12.7%.8,9 This latter condition requires the execution of

clinical-instrumental investigations such as ultrasound and mammography in order to exclude the coexistence of an otherwise undiagnosed breast cancer. In literature, the reported cases mainly result from surgery or biopsy for breast cancer, mastoplasty, mastopexy or cases associated with other diseases.10,11 The diagnosis is not difficult and it is predominantly clinical. The course is usually benign. We report here six new cases of MD caused by different factors that came to our attention by other diseases.

Method and results Patient 1 was a 16-year-old female patient with a considerable development of musculoskeletal system in relation to sex and age (smoker, no thrombosis or thrombophlebitis in the patient’s family history and Department of Surgical Sciences, Sapienza University of Rome, Rome, Italy Corresponding author: Valerio D’Orazi, Department of Surgical Sciences, Sapienza University of Rome, Viale Regina Elena 324, 00161 Rome, Italy. Email: [email protected]

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no use of drugs). After admittance to our department, the patient reported that, following an intense workout, a hard and painful cord appeared proximal to the left nipple in the direction of the left midclavicular area, as seen on physical examination. No breast lump was palpable and ultrasonography was negative. After the visit, given anamnesis and the young age of the patient, no other investigations were performed and MD was diagnosed. Therapy with non-steroidal anti-inflammatory drugs (NSAIDs) for local use was recommended. Twenty days later, the patient showed complete regression of clinical signs and the hemocoagulation markers and enzymes performed after healing were normal. A subsequent breast ultrasonography allowed us to exclude further breast disease. Patient 2 was a 42-year-old female patient who reported the presence of a hard and painful reddened stria that distally extended from the areola to the right chest wall. This reddened stria apparently developed after a hike with a backpack on her shoulders. The physical examination revealed that the traction of nipple–areola complex caused retraction of the overlying skin. Even in this case, MD was diagnosed and given the presence of inflammation and pain, antibiotic therapy (azithromycin dihydrate 500 mg/day past ovulation (PO) for three days) and topical application of NSAIDs were suggested. Blood tests confirmed normal hemocoagulation factors and instrumental examinations (i.e. ultrasonography and mammography) excluded the presence of additional breast disease. Four weeks later, she recovered from the disease without any symptom and achieved a complete restitutio ad integrum. Patient 3 was a 49-year-old obese female patient who reported a slow and gradual volume increase of both breasts during the last year, in the absence of hormonal changes worthy of note, except a mild hypothyroidism treated with hormone therapy. About 20 days before coming to our observation, the patient found a cutaneous retraction of the left breast (Figure 1(a)) with redness likely due to the presence of thrombophlebitis of a large superficial vein. The patient refused examination and 15 days after symptom onset, she reported the formation of a large hematoma at the first bifurcation of that vein which subsequently became infected, causing a big breast. At this point, the patient came to our outpatient observation and surgical treatment. We found thrombophlebitis of a large superficial vein, with a longitudinal course between the upper quadrants of the left breast (Figure 1(b)), and the big breast abscess that was immediately drained (about 500 cc of pus mixed with blood). The bacterial culture showed prevalence of Proteus mirabilis. In addition to antibiotic coverage (amoxicillin 1 g/12 h PO for eight days), the treatment consisted in the drainage of the

abscess cavity and its daily irrigation, taking care to leave in place a laminar drainage which was replaced from time to time. After achieving healing, magnetic resonance imaging with contrast agents was performed to exclude the coexistence of breast cancer. Subsequently, the patient underwent bilateral breast reduction surgery, as reported for gigantomastia,12,13 removing approximately 3500 g of left breast tissue and 2500 g of right breast tissue, to prevent that the breast’s weight and the consequent stretching of the mammary veins could cause recurrence. Three years after treatment, the patient showed no signs of residual disease. Patient 4 was a 42-year-old female patient who underwent four hormonal stimulation for in vivo fertilization (IVF) after which the patient had a pregnancy. The hemocoagulation tests were normal. Six months after breast-feeding, the patient revealed the presence of a nodule (approximately 1.5 cm, hypoechoic) in the upper lateral quadrant of the right breast. Following its rapid volumetric increase, the patient underwent diagnostic lumpectomy that confirmed to be adenosis with apocrine metaplasia and fibroadenomatoid hyperplasia. As we usually do, we recommended the patient to wear an elastic bra in the postoperative. The too eager patient wore a particularly tight-fitting one, even during the night. Seven days after surgery, the patient reported that she noticed the presence of a cord-lesion extending from right breast to the thoracoabdominal region (Figure 2). After clinical examination, we diagnosed MD and suggested not to wear the constricting garment that likely worsened the MD and to locally apply a ketoprofen-based ointment. Three days after the beginning of therapy, the lesion was significantly reduced and healed. Patient 5 was a 63-year-old Caucasian female patient, in treatment with anti-platelet therapy (acetylsalicylic acid 100 mg/day) for a previous thrombosis of left retinal vein. About one year before coming to our observation, the patient underwent surgery for left breast carcinoma (Upper-Outer Quadrantectomy and sentinel lymph node excision). Histopathology showed high-grade invasive ductal carcinoma (pT1b, pN0—G3—ER 90%, PR 80%, Ki67 13%—Negative sentinel lymph node). The patient was subsequently treated with radiotherapy and anastrozole-based hormonal therapy. One year after surgery, following transport a luggage, the patient noticed the presence of a retracted cord-tumefaction nearby the surgically treated axilla (Figure 3). Therefore, she came to our outpatient surgery center and after complete history and physical examination a diagnosis of MD was done. We recommended local anti-inflammatory therapy with diclofenac, recommending not to suspend the ongoing anti-platelet therapy. About 20 days later, the lesion

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Figure 1. (a) Clinical finding of the patient with visible cord and retraction (black arrows) at the upper quadrant of the left breast that showed (b) thrombophlebitis of a large superficial vein (with arrow) with a longitudinal course between the upper quadrants of the left breast.

Figure 3. Clinical finding of the patient. Visible retracted cord-tumefaction nearby the surgically treated axilla.

Figure 2. Clinical finding of the patient showing the cord-lesion extending from right breast to the thoracoabdominal region (arrows).

shrank approximately 70% and therefore, antiinflammatory therapy was confirmed for a further month. Patient 6 was a 78-year-old Caucasian male patient, in treatment with low-molecular weight heparin for

previous acute myocardial infarction (AMI), undergoing mastectomy at the right breast with axillary lymphadenectomy first and second level, for an infiltrating ductal carcinoma of intermediate grade (G2), pT4 N0, ER 85%, PR 75%, Ki67 13%, negative lymph nodes. About 15 days after surgery, following a routine visit, a hard cord was visible at the right axilla (Figure 4), compatible with MD. The patient was then treated with local ketoprofen until healing of the lesion and maintenance of anti-coagulant therapy.

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Figure 4. Clinical finding of the patient showing the retracted cord-tumefaction nearby the surgically treated axilla.

among subacute vasculitis, cutaneous polyarteritis nodosa and drugs-induced vasculitis (mainly penicillins, sulfonamides, thiouracil, iodides and allopurinol). Those conditions are typical of particularly painful inflammatory diseases and they affect the arteries more than veins. The treatment of MD is mainly symptomatic, based on the administration of NSAIDs and analgesics or sometimes by perilesional anesthetic infiltration, as reported by some authors.9,19,20 When treatment is not sufficient to solve the symptoms or in case of relapse, a thrombectomy or resection of the affected vein can be performed.2,4 Furthermore, it hass not been proven that MD is a risk factor for thrombophlebitis in other anatomic sites.

Conclusions Discussion The MD is a relatively rare infection of the breast. In the literature, only few authors reported an incidence rate between 0.5% and 0.8%.14,15 In our opinion, this incidence is not correctly estimated because the syndrome is not well known and therefore, is not always correctly identified. As a matter of fact, we describe here six cases of MD that underwent our outpatient observation in the past four years. Although the MD may be associated with numerous pathological conditions such as accidental trauma, muscle strains, bacterial and viral infections, gigantomastia and/or drug treatments,3,6 to date no specific etiopathogenic factors have been identified. Thus, in the literature only MD cases resulting from complications of surgery16,17 and/ or concomitant diseases have been reported.10,11 In addition, the MD cases are often treated as outpatients even by non-specialist doctors (which, in our opinion are the most numerous) who fail to report them in the literature and therefore, do not fall under case studies. Fortunately, the pathology can be considered benign and self-limiting; it spontaneously resolves in two to four months and has a favorable prognosis.2 However, considering the hypothetical coexistence of a neoplastic disease of which the MD could be the warning light, or considering the other systemic diseases that often cause MD,8,11,18 it is good practice to perform all clinical and instrumental tests. The diagnosis is essentially clinical, but ultrasonography or a Doppler ultrasound,2 as well as all the clinical/instrumental tests can also be performed that are routine in the early diagnosis of breast cancer or other cancers (e.g. phlebitis migrant), to exclude the presence of a tumor as a contributory cause of the disease. In the case of MD, mammography reveals a truncal density superficially localized, corresponding to the palpable lesion. The differential diagnosis must be made

We report here six new cases of MD. In two cases, the MD was caused by compression, one by hypertrophy of musculoskeletal system for intense sport activity and the second one by a backpack strap. The third case was caused by the stretching of the dorsal vein of the breast due to the weight of gigantomastia, the fourth case was subsequent to hormonal stimulation for IVF, while the fifth and the sixth cases (one of them was a male) were patients who underwent surgery for breast carcinoma, both in therapy with anti-coagulant drugs (one for increased risk of thrombosis and one for previous AMI). In agreement with the literature reported up to now, we found that MD can be caused by extremely different factors and although it is possible to identify the causes, these are not predictable nor preventable. Given that MD is considered a rare and usually self-limited benign disease whose diagnosis and treatments, ranging from therapeutic abstention, anti-inflammatory drugs or even surgery, are debated, the description of new cases might be helpful to the scientific community to reach a future consensus.

Authors’ contributions VP, PU and VDO designed and wrote the paper. VP and VDO performed the surgery. DS, LDV and AP performed the literature search and supported the writing of the paper. All authors read and approved the final manuscript.

Consent Written informed consent was obtained from the patients for publication of this manuscript and any accompanying images. A copy of the written consent is available for review by the Editor-in-Chief of this journal.

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Conflict of interest None declared.

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Funding This research received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

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