Abstract
Introduction:
Hydatid disease of the male breast has not been documented in literature to date.
Case report:
We present a case of a 46-year old male patient who presented with a painful breast lump for 1 year. On imaging, it was suspicious for hydatid disease of the breast and was successfully diagnosed preoperatively on fine-needle aspiration cytology and histopathology as hydatid disease of the breast.
Discussion:
Incidence of hydatid disease of the breast contributes to a minute number of total cases of hydatid disease. Male breast hydatid disease was unknown prior to this report. We did find similarities in the imaging features of our case and the ones described for female breasts. On ultrasonography, we found it analogous to the ‘water-lily’ sign which has been described in hepatic hydatic cysts.
Conclusion:
Keeping hydatid disease as a differential in the setting of a suspicious breast mass even in a male patient proved to be rewarding as it negated the need for further cross-sectional imaging and a definitive diagnosis could be sought based on the fine-needle aspiration cytology and histopathological findings.
Introduction
Breast as a site for involvement in hydatid disease is rare and accounts for less than 0.3% of all the cases of the disease. 1 Larva of the Echinococcus granulosus or less frequently that of Echinococcus multilocularis causes hydatid disease. It has myriad ways of presentation and the symptoms depend on which organ of the body is involved. The most frequent initial complaint in cases with hydatid disease of the breast is a localized painless swelling. The lifecycle of E. granulosus usually involves an intermediate and a definitive host. Carnivorous animals are considered to be the definitive hosts and the herbivores as the intermediate hosts. 2
Usually, humans just serve as an unintentional host; the main hosts are dogs, sheep and so on. 3 However, in a situation where the parasite is ingested by contaminated food, the eggs hatch into oncospheres and enter the portal and lymphatic circulation via the gastrointestinal tract. They then develop into larvae within viscera.
Case report
A 46-year old male, an auto rickshaw driver by profession presented with a tender breast lump in the right breast for 1 year. The lump had increased in size over the duration and the overlying skin was becoming increasingly sensitive. There was no history of nipple discharge, fever, past intervention, local trauma or animal bite to suggest direct implantation of the parasite. However, there was history of contact with dogs as the patient has always had a pet dog for the past 8 years. On examination, there was a tender lump in the right chest involving the nipple areolar complex region (Figure 1).
Inflamed right breast lump involving the nipple areolar complex.
Ultrasound (USG) of the lump was advised as a first investigation as it is the institute protocol in a breast lesion. The examination was conducted using a high-frequency linear transducer (10–15 MHz) set at depth of 4 cm which revealed a thick walled oval cystic lesion (Figure 2) of size 2.26 × 2.68 × 2.41 cm with a volume of 7.6 mL in the retro areolar region of right breast extending from 4 to 5 o’clock position. On USG two walls of the lesion, the outer wall (pericyst) (Figure 3) was identified and was composed of normal breast tissue; it measured 0.19 cm with free floating internal curvilinear membranes representing the detached endocyst membrane (inner wall). No internal vascularity was seen on colour Doppler (Figure 4). Both breasts were scanned for similar lesions and axillae for lymphadenopathy but was negative. Internal curvilinear septae like structures on USG was visualized and a diagnosis of a complex infected cystic lesion was made with hydatid cyst as the first differential.
Thick walled oval cystic lesion in the retro areolar region of right breast extending from 4 to 5 o’clock position.
Outer wall of the lesion (pericyst) which is was composed of normal breast tissue with floating internal curvilinear membranes which represents the detached endocyst membrane (water-lily sign).
Absent vascularity on colour Doppler.
On USG, it resembled the typical water-lily sign, 4 also known as the camalote sign where there is a detachment of the endocyst membrane which results in floating curvilinear membranes within the pericyst that resemble the appearance of a water lily. Chest X-ray and an USG abdomen were performed to rule out other areas of involvement and were negative. A USG-guided fine-needle aspiration cytology (FNAC) was further advised as it was an atypical appearance for a complex infected cystic lesion, the result was inconclusive revealing only chronic inflammation.
On strong radiological suspicion, cell block from the cyst fluid revealed multiple protoscolices of E. granulosus (Figure 5) and acellular lamellated membrane clinching the diagnosis of breast hydatid disease (Figure 6). After treatment with Albendazole, the size reduced and the cyst was completely excised with no rupture or spillage. There was no recurrence on the last follow-up of the patient 6 months after surgery.
Protoscolices with sucker and refractile hooklets (hemtoxylin and eosin, 400×).
Acellular lamellate layer (hemtoxylin and eosin, 400×).
Discussion
The breast can be the only site or a part of disseminated hydatid disease. It is described in literature only in female patients, and it presents as a painless breast lump that slowly increases in size, and when secondary infection exists, the lesion is clinically indistinguishable from a breast abscess. 5
The incidence of the infection is higher in sheep rearing, developing and underdeveloped Mediterranean countries, South America, the Middle East, Asia and East Africa.3,6 It generally affects women aged 30–50 years, although a wider age range of 26–74 years has been reported.6,7 Clinically, a hydatid cyst in the female breast might mimic fibroadenomas, cystic mastopathies, phyllodes tumours, chronic abscesses or even carcinomas. For a brief period, the diagnosis of hydatid cyst based on FNAC was contraindicated in lieu of the anaphylactoid reactions; however, they have proven to be of substantial benefit. 8
There is no conclusive explanation in the literature for females being affected more than males; however, we feel that the larger surface area of lymphatic channels in the female breast may be a contributory factor. USG has been deemed the gold standard for diagnosing, staging and follow-up for breast hydatid disease with a sensitivity of 88%–98% and a specificity of 95%–100%. 7
A chest radiograph and USG abdomen are considered useful additional investigations utilized commonly to rule out disseminated hydatid disease. 8 We ruled out direct implantation of the parasite from the negative history; therefore, the only plausible route of infection would be faecal-oral which is supported by the long history of having a pet dog.
Computed tomography (CT) is the imaging modality of choice to diagnose and characterize lung hydatid cysts. Whereas both CT and magnetic resonance imaging (MRI) are important for evaluation and classification of liver hydatid cysts as well as the diagnosis of central nervous system and musculoskeletal hydatid cysts. On MRI, the findings of a cystic lesion with enhancing capsule are suggestive of hydatid cyst. They will appear as any cystic lesion-hypointense on T1-weighted images (T1WIs) and hyperintense on T2-weighted images (T2WIs). However, a low signal-intensity rim ‘rim sign’ is more evident on T2WIs. Daughter cysts may appear hypointense or isointense relative to the maternal matrix on T1WIs and T2WIs. The ‘serpent sign’ or ‘snake sign’ which represents collapsed membranes of low signal intensity in all sequences is another specific imaging feature of a hydatid cyst. 9
The presence of hydatid cysts’s occasional thin calcified coat and a thicker, more laminated wall may help in differentiating it from a simple cyst. Ultrasonography as a modality has the ability to visualize floating membranes, daughter cysts and vesicles. Although in the liver Gharbi et al. 10 described five types of cysts in 121 patients the types being (1) unilocular cyst with thick wall; (2) cyst with detached germinative membrane; (3) multicystic appearance with internal septations; (4) degenerative pseudo-solid cysts and (5) solid content with calcified wall. This classification has been widely used even in the extrahepatic hydatid cysts as well. This classification was further refined by a World Health Organization (WHO) informal working group on echinococcosis to include fertile, transitional and inactive cysts. 11 According to the 2001 WHO classification of hepatic hydatid cysts, we feel this is a breast analogous of the CE 3A (transitional stage) 3A where the daughter cysts have detached laminated membranes within the encompassing parent cyst (water-lily sign).
Depending on the cyst type, treatment options include complete surgical removal or medical and other percutaneous interventional methods. Another pattern, the ‘congealed water-lily sign’, in which the hydatid fluid transforms from a watery gel to a viscid one, was identified by Durr-e-Sabih et al. Wherein curvilinear structures and intact folds of the germinal layer that were previously trapped within the viscid matrix now become visible as a result of this transformation. They added that this sign strongly suggested the presence of hydatid cysts. 12
The USG-guided FNAC is a preoperative diagnostic tool that can provide a safe, quick, affordable and accurate diagnosis.13,14 Protoscolices, hooklets and laminated membranes can be identified without complications. The treatment of the breast hydatid cyst is cystectomy with pericystectomy to protect rupture and reinfestation in addition to Albendazole for further prevention. 15 The history of past intervention is important as pointed by Gomori et al., 16 wherein the appearance of the ‘water-lily’ sign was visualized after an attempted cyst puncture, which means a cyst in the active phase may also appear to be in the transitional stage if puncture/FNAC was attempted previously.
Conclusion
Hydatid disease of the female breast is an extraordinary location of this disease, and this is the first documented case in a male breast. It is wise to have this entity in the differential list especially when dealing in the endemic areas. We were able to successfully come to a preoperative diagnosis which is usually not the situation; however, in our case, as it was in a regenerative stage with the ‘water-lily’ appearance which was relatively facile to identify. An accurate preoperative diagnosis goes a long way in preventing unprecedented intraoperative rupture.
Footnotes
Contributors
Dr H.S.S.: substantial contributions to conception and design, or acquisition of data or analysis and interpretation of data. Dr S.M.: substantial contributions to conception and design, or acquisition of data or analysis and interpretation of data. Dr A.P.: substantial contributions to conception and design, or acquisition of data or analysis and interpretation of data. Dr C.M.: substantial contributions to conception and design or analysis and interpretation of data.
Declaration of conflicting interests
The author(s) declared no potential conflicts of interest with respect to the research, authorship and/or publication of this article.
Funding
The author(s) received no financial support for the research, authorship and/or publication of this article.
Ethics approval
Waived off by the Institutes Ethics body of Institute of Medical Sciences & SUM Hospital – Faculty of Medical Sciences as it is a case report on 20 January 2023 in the name of Dr H.S.S.
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