DIAGNOSTIC DILEMMAS Section Editor: Claire W. Michael, M.D.
Gigantomastia in Pregnancy With an Accessory Axillary Mass Masquerading as Inflammatory Carcinoma M. D. S. Lokuhetty, M.B.B.S., Dip. Path., M.D. Histopathology, M.I.A.C., P. A. M. Saparamadu, M.B.B.S., Dip. Path., M.D. Histopathology, D. M. A. Al-Sajee, M.B.Ch.B., M.Sc. Histopathology,* and R. Al-Ajmi,
Giagantomastia is a rare disorder known to occur in pregnancy, causing enlargement of the breasts greater than that of gravid enlargement. The histological features of gigantomastia are glandular hyperplasia and an increase of stromal tissue. Illustrated by one documented case, cytomorphology of gigantomastia was misdiagnosed as a phyllodes tumor. We document the cytomorphology of an axillary mass in a gravid woman of 24 years with gigantomastia. She presented in her first trimester with bilateral mastalgia and swelling, nonresponsive to antibiotics. Imaging excluded mass breast lesions and a pituitary prolactinoma. The breasts progressively enlarged, became warm, tender, and developed skin ulcerations and a peau d’orange appearance. Subsequently she developed a mass in her left axilla. On aspiration of the mass, some of the cytomorphological features were suspicious for a metastasis, which correlated well with her clinical features. Careful evaluation suggested cytomorphology to be compatible with benign accessory breast tissue with possible hormone related changes of pregnancy. Histology of the excised axillary mass confirmed this diagnosis. Thus, awareness of this rare condition and careful evaluation is mandatory to avoid misdiagnosis in a similar clinical context. Diagn. Cytopathol. 2011;39:141–143. ' 2010 Wiley-Liss, Inc. Key Words: gigantomastia; axillary breasts; cytomorphology, inflammatory carcinoma
Gigantomastia in pregnancy or gravid macromastia is a rare disorder of undetermined etiology. It leads to diffuse, massive breast hypertrophy exceeding the usual physioDepartment of Pathology, Sultan Qaboos University Hospital, Muscat, Oman *Correspondence to: D. M. A. Al-Sajee, M.B.Ch.B., M.Sc. Histopathology, Department of Pathology, Sultan Qaboos University Hospital, PO Box 38, Post Code 123, Al Khoud, Muscat, Oman. E-mail:
[email protected] Received 23 November 2009; Accepted 17 February 2010 DOI 10.1002/dc.21393 Published online 24 May 2010 in Wiley Online Library (wileyonlinelibrary.com). '
2010 WILEY-LISS, INC.
M.D.
logical gravid enlargement. The pathogenesis of this condition is unknown and is postulated to be due to hormonal excess and/or hypersensitivity of the target organ. Histology of gigantomastic tissue has shown glandular hyperplasia and abundant stromal tissue1 with acinar and periacinar stromal fibrosis.2 The cytomorphological appearance of gigantomastia is hitherto under described. The cytological smears of the single documented case had shown spindled stromal cells leading to a misdiagnosis of a phyllodes tumour.2 We aim to document the cytomorphological appearance of a unilateral axillary mass in a gravid woman with bilateral gigantomastia, whose clinical presentation masqueraded as inflammatory carcinoma3 with axillary metastasis. The diagnostic dilemma faced in this clinical context during cytomorphological diagnosis will be discussed.
Case Report A 24-year-old female presented with bilateral mastalgia and swelling, suggesting inflammatory mastitis in the 4th month of the second pregnancy. She was treated with antibiotics with no response. Radiological studies were negative for mass breast lesions and a pituitary prolactinoma. The breasts were massively enlarged at 6 months of gestation (right breast—60 cm horizontally at nipple level and 49 cm longitudinally at mid clavicular line, left breast—69 cm horizontally at nipple level and 49 cm longitudinally at mid clavicular line) and were warm and tender with healing ulcers and skin peau d’orange. The nipples were normal. She subsequently developed a large left axillary mass (10 3 10 cm) raising the possibility of Diagnostic Cytopathology, Vol 39, No 2
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Diagnostic Cytopathology DOI 10.1002/dc
LOKUHETTY ET AL.
Fig. 1. Moderately enlarged epithelial nuclei with anisokaryosis and hyperchromasia, with no evidence of nuclear membrane irregularity, coarse chromatin, and nucleoli. Papanicolaou, 6003. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary. com.]
Fig. 2. Bare oval nuclei and discohesive epithelial cells with intact cytoplasm in a background of proteinaceous fluid admixed with blood. H&E, 6003. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
inflammatory carcinoma with axillary metastasis during gestation. Aspiration smears from the axillary mass comprised of a few cohesive clusters of duct epithelial cells with a moderate amount of cytoplasm. The nuclei were moderately enlarged with anisokaryosis and hyperchromasia with no evidence of nuclear membrane irregularity, coarse chromatin or nucleoli (Fig. 1). The background contained a few bare oval nuclei and discohesive epithelial cells with an intact cytoplasm. It also contained proteinaceous fluid and blood (Fig. 2). A few epithelial nuclei stripped of cytoplasm were seen in other areas (Fig. 3). The cytomorphological appearance was reported as benign, in keeping with accessory breast tissue. Subsequent develop142
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Fig. 3. Epithelial nuclei stripped of cytoplasm in a background of proteinaceous fluid. Papanicolaou, 6003. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
Fig. 4. Hyperplastic terminal duct lobular units in oedematous stroma. H&E, 403. [Color figure can be viewed in the online issue, which is available at wileyonlinelibrary.com.]
ment of a massive haematoma at the aspiration site led to the excision of the axillary mass. Histological examination of the mass confirmed it as benign accessory breast tissue affected by gigantomastia with glandular hyperplasia and oedematous stroma (Fig. 4). Acinar and periacinar fibrosis were absent. She delivered a live baby by caesarian section at 38 weeks of pregnancy. The reduction in breast size was minimal when she was reviewed at 7 weeks post partum, before she was lost to follow up.
Discussion Gigantomastia is rare in pregnancy with a documented incidence of approximately 1:100,000 pregnant women.4 It usually affects both breasts, however, on occasions be
Diagnostic Cytopathology DOI 10.1002/dc
GIGANTOMASTIC AXILLARY BREAST: CYTOMORPHOLOGY
unilateral, affecting a single breast.2,5 Gigantomastia of both breasts with enlarged accessory axillary breast tissue presenting as bilateral axillary masses is even rarer.6 Women with gigantomastia develop axillary masses when the same process affects accessory axillary breast tissue. Majority of women affected by gigantomastia are primiparous and the condition could recur in subsequent pregnancies once it is established. Mastectomy or breast reduction is usually undertaken following delivery to overcome the incapacitating effects of the condition.7 Inflammatory breast cancer is characterized by rapid onset of breast warmth, erythema, and oedema without a well defined breast mass. The breasts may also enlarge to two or three times its normal volume within a few weeks. Affected patients often have early involvement of axillary lymph nodes.3 Thus, bilateral gigantomastia with skin changes and a unilateral axillary mass could clinically masquerade inflammatory carcinoma with axillary metastasis. Aspiration smears of the axillary mass of this gravid woman showed discohesive epithelial cells with intact and stripped cytoplasm and moderately enlarged epithelial nuclei with anisokaryosis and hyperchromasia. These cytomorphological features were suspicious of possible axillary metastasis. However, other malignant cytological features such as nuclear membrane irregularity, coarse chromatin, or nucleoli were absent. Bare oval nuclei were present in the background. The lymphoid background expected from an axillary node was absent. These cytomorphological features and the possibility of gravidic gigantomastia with enlarged accessory axillary breast tissue, dissuaded a suspicious or malignant cytomorphological diagnosis in this case. The nuclear changes of enlargement, anisokaryosis, hyperchromasia, stripped epithelial nuclei, and proteinaceous material seen in the back ground of these smears could be attributed to hormone related changes of the duct and acinar epithelium of accessory breast tissue during pregnancy.8 The stromal spindle cells that led to the
diagnosis of a phyllodes tumour in the single documented case were absent. The aspiration smears of gigantomastia could contain spindle stromal cells without epithelial cells if only the prominent stroma is sampled. This could be a potential pitfall. In conclusion, the cytomorphology of gravid gigantomastia, could be misleading especially when it masquerades as inflammatory carcinoma with axillary node metastasis as in this case. Careful cytomorphological evaluation and awareness of this rare condition in pregnancy avoided misdiagnosing it as a possible axillary metastasis.
Acknowledgments The technical support extended by Ms. Usha Rani Bai, Senior Biomedical Scientist and Ms. Maria Virginia T. Uy, Biomedical scientist of the Department of Pathology, Sultan Qaboos University Hospital, Oman, is gratefully acknowledged.
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