Tumori, 94: 762-764, 2008
A rare cause of mastalgia: Dercum’s disease (adiposis dolorosa) Chiara Trentin1, Brunella Di Nubila1, Enrico Cassano1, and Massimo Bellomi2,3 1
Breast Imaging Unit, Department of Radiology, European Institute of Oncology, Milan; School of Medicine, University of Milan, Milan; 3Department of Radiology, European Institute of Oncology, Milan, Italy
2
ABSTRACT
Among the symptoms reported by patients undergoing breast imaging, pain is one of the most common. Mastalgia is generally classified as cyclic, noncyclic, or extramammary. A rare cause of breast pain is Dercum’s disease, or adiposis dolorosa, a condition characterized by multiple, often painful subcutaneous lipomas. Painful breast lipomas have not been described in the literature so far. The diagnosis is made by clinical means supported by diagnostic imaging. We report the case of a 58-yearold postmenopausal woman affected by Dercum’s disease in the breast. Ultrasound examination showed multiple oval, well-defined, hyperechoic lesions suggestive of breast lipomas. No significant features were detected by mammography.
Introduction Dercum’s disease, or adiposis dolorosa, is a condition of unknown etiology characterized by multiple, often painful subcutaneous lipomas. It was first described in 1892 by FX Dercum as a rare syndrome that principally affected overweight, postmenopausal women1. A few cases of male patients and some familial forms have been reported2. Painful lipomas can be located in any fat tissue; some authors described cases at articular or pelvic sites where the pain affected the quality of the patients’ life1.
Material and methods We report the case of a 58-year-old obese woman (who had been on antidepressant therapy for 4 years) affected by breast localizations of Dercum’s disease. The patient was referred to our institute for a breast ultrasound examination after a negative mammography. She complained of severe pain in circumscribed areas of the breast and in the right arm, where a 3-cm elastic subcutaneous mobile tumor was present. The arm pain had started 6 months before, while the breast pain had started 2 years before and had increased in the last 2 months to such intensity as to hamper sleep in the prone position. Ultrasound examination was performed with a 10 MHz linear probe using Technos equipment (Esaote, Genoa, Italy). At both sites the pain was focalized and continuous and it was exacerbated by the pressure of the probe.
Results The preliminary clinical breast exam did not show any significant findings. During palpation, the pain was felt at both upper outer quadrants, but it reached unbearable levels only at specific points. The previous mammogram had shown a mixed breast structure (partially glandular and partially fatty) with benign calcifications but no
Key words: mastalgia, breast pain, Dercum’s disease, adiposis dolorosa, painful lipomas. Correspondence to: Chiara Trentin, MD, Breast Imaging Unit, Department of Radiology, European Institute of Oncology, Via Ripamonti 435, 20141 Milan, Italy. Tel +39-02-57489070; fax +39-02-57489040; e-mail
[email protected] Received March 30, 2007; accepted July 11, 2007.
A RARE CAUSE OF MASTALGIA: DERCUM’S DISEASE
suspicious findings. In correspondence with the most painful areas, ultrasound examination showed multiple hyperechoic oval lesions with well-defined margins and a diameter ranging from 8 to 21 mm but no signs of vascularization on color Doppler (Figure 1). In the volar region of the right arm there was a 31-mm, oval, isoechoic lesion with no vascularization (Figure 2). The ultrasound findings were compatible with lipomas.
Discussion From puberty to menopause, mastalgia is one of the most frequent complaints reported by women3. Its intensity changes from slight or moderate to severe (about 2-5% of all forms), causing insomnia and many impediments to normal physical and sexual activities.
Figure 1 - Breast ultrasonography of the reported case: oval homogeneous hyperechoic lesion with well-defined margins and no vascularization at color Doppler. The picture is compatible with a breast lipoma.
Figure 2 - Ultrasound of the right arm: oval homogeneous isoechoic lesion with well-defined margins and a thin hyperechoic peripheral capsule, without vascularization at color Doppler.
763
Cyclic mastalgia is the most common type reported in women in their thirties and is strictly related to the menstrual cycle and correlated with endocrine alterations. It has a variable time span with an apex in the premenstrual phase; the pain may be unilateral or bilateral (commonly in the upper-external region radiating to the ipsilateral axilla and arm) but is not precisely localized. Noncyclic mastalgia is less frequent (30%), it is not related to the menstrual cycle and affects women in their forties. The pain is often well localized, unilateral, and intermittent with continuous exacerbation. Generally it is idiopathic or related to the presence of benign lesions such as cysts or duct ectasia4,5. Finally, the cause of breast pain may be Tietze’s syndrome, characterized by continuous superficial pain aggravated by chondrosternal pressure; the pain is localized to the inner breast regions3. Dercum’s disease may be included in the group of noncyclic mastalgia. The disease is hereditary with an autosomal dominant pattern showing variable penetrance; the etiology is unknown6,7. Most cases are sporadic, although cases of affected siblings have been reported. Postmenopausal women are most commonly affected, while very few cases have been reported in men. The clinical symptoms are nonspecific: patients often have multiple lipomas in the subcutaneous tissue of the knees, back, neck, thighs, or arms, while the face and hands remain unaffected. The lipomas are spontaneously painful, with paresthesia in the overlying skin. The appearance of the skin is normal or shiny and tense with dilated superficial veins. Asthenia and fatigue as well as a low pain threshold seem common in patients with Dercum’s disease. Emotional lability, irritability, depression, epilepsy, confusion, and dementia are often present and complicate the diagnosis. Lipomas at the joints cause mechanical arthralgia8. Septic shock was described in the literature as a rare complication following steatocutaneous necrosis of a large, superficial, fatty tumor9. Imaging is useful to confirm the clinical suspicion in the presence of a palpable lesion, or to obtain a diagnosis in case of a nonpalpable lesion. The diagnosis is made by ultrasound in superficial lesions (skin, muscles, and breast) or by magnetic resonance imaging if the pain has a deep origin (joint, abdomen)1. In the case described here, ultrasound imaging enabled us to make the diagnosis both in the breasts and arm. Lipomas present with an ultrasonographic pattern of fatty tissue, an oval or lenticular shape, and well-defined margins. They are also easily compressible by the probe. The internal texture is homogeneous, isoechoic, hyperechoic or hypoechoic (Figure 3). Lipomas of the same echogenicity as the surrounding fatty tissue are very difficult to recognize, unless they are large enough to displace the normal structures or have a peripheral calcified capsule10. Echo-poor lipomas are
764
Figure 3 - Breast ultrasound: oval homogeneous isoechoic lesion with well-defined margins and a thin hyperechoic peripheral capsule associated with vascularization at color Doppler.
visually almost identical to fibroadenomas; mammograms are useful to confirm the diagnosis because fibroadenomas have soft tissue density while lipomas are radiolucent with occasional peripheral ringlike calcifications (Figure 4)11.
C TRENTIN, B DI NUBILA, E CASSANO, M BELLOMI
Two treatment options are described in the literature: surgical and medical. Surgical treatment is based on lipoma excision or on liposuction. The pain ceases almost immediately, but there are frequent relapses that may be identified by imaging. Medical therapy consists of repeated intravenous administration of lidocaine, with complete pain relief occurring within 10 hours to 12 months after treatment9-12. No treatment has been performed in our patient so far. As far as we know, there are no previously reported cases of Dercum’s disease of the breast. Nevertheless, considering that breast lipoma and breast pain are both common, may be associated, and tend to be given limited clinical attention as they do not imply major problems of differential diagnosis with cancer, such cases could be more frequent than it appears from the literature. Breast pain is a highly frequent complaint in women. It is generally not associated with breast cancer. The radiologist should perform accurate examinations (such as mammography and ultrasound) to exclude suspicious features and reassure the patient. We should not forget that ultrasound allows the diagnosis of painful lipomas that are responsible for mastalgia in rare cases of Dercum’s disease.
References
Figure 4 - Right craniocaudal mammogram showing a large, sharply-outlined radiolucent mass with a thin radiopaque capsule in the lateral region.
1. Harris K, Davies K, Dumont S, Stephenson BM: A pain in the groin. Lancet, 350: 334, 1997. 2. Solivetti FM, Thorel MF, Marandino F: Breast lipomas in echography. A discussion of 3 cases and a review of the literature. Radiol Med, 99: 281-282, 2000. 3. Cataliotti L, Bianchi S, Ciatto S: Patologia mammaria benigna. In: Senologia oncologica, Veronesi U (Ed), pp 177178, Masson, Milano, 1999. 4. Peters F, Diemer P, Mecks O, Behnken L LJ: Severity of mastalgia in relation to milk duct dilatation. Obst Gynecol, 101: 54-60, 2003. 5. Santen RJ, Mansel R: Benign breast disorders. N Engl J Med, 353: 275-285, 2005. 6. Amine B, Leguilchard F, Benhamou CL: Dercum's disease (adiposis dolorosa): a new case-report. Joint Bone Spine, 71: 147-149, 2004. 7. Campen R, Mankin H, Louis DN, Hirano M, Maccollin M: Familial occurrence of adiposis dolorosa. J Am Acad Dermatol, 44: 132-136, 2001. 8. Defranzo AJ, Hall SH, Herring SM: Adiposis dolorosa (Dercum’s disease): liposuction as an effective form of treatment. Plast Reconstr Surg, 85: 289-292, 1990. 9. Haddad D, Athmani B, Costa A, Cartier S: Dercum’s disease: a severe complication in a rare disease. A case report. Ann Chir Plast Esthet, 50: 247-250, 2005. 10. Rizzatto G, Chersevani R, Giuseppetti GM: Mammella. In: Trattato italiano di ecografia, Vol 2, Solbiati L (Ed), p 574, Poletto Edizioni, Milano, 1993. 11. Muttarak M, Chaiwun B. Imaging of giant breast masses with pathological correlation. Singapore Med J, 45: 132139, 2004. 12. Devillers AC, Oranje AP: Treatment of pain in adiposis dolorosa (Dercum’s disease) with intravenous lidocaine: a case report with a10-year follow-up. Clin Exp Dermatol, 24: 240-241, 1999.
