A 48-year-old female with no known comorbidities presented to our hospital with chief complaints of hemoptysis, intermittent headache, hoarseness of voice with difficulty in speech and pain in the throat, and bilateral progressive hearing loss. She also had a painless lump in the right breast, bilateral lower limb swelling with purple-colored spots over the legs. However, there was no history of fever, cough, breathlessness, or chest pain.
On physical examination, her vitals were found to be within normal limits. There was bilateral pitting pedal edema with palpable purpura over the bilateral lower limbs (Fig. 1). In addition to this, there was a palpable lump in the right breast; however, the rest of her systemic examination was normal.
On routine blood investigations, the total cell count was elevated with a predominance of neutrophils (76%). Her erythrocyte sedimentation rate (89 mm/h), C-reactive protein (46.8 mg/L) and D- Dimer values (4.6 μg/ ml) were elevated. The liver and renal function tests were found to be within normal limits. The blood and urine cultures were negative and urine analysis was positive for erythrocytes and showed hyaline casts.
Given the history of hoarseness of voice and difficulty in hearing, the patient was referred to the ENT department. A video laryngoscopy was done, which revealed left vocal cord palsy with pooling of saliva in the left pyriform fossa (Fig. 2). Bilateral otitis media was also noted at the same time.
A contrast-enhanced computed tomography (CECT) of the thorax was arranged because of elevated D-dimers and hemoptysis and it revealed few well-defined, heterogeneously enhancing lung parenchymal lesions of varying sizes and the largest of these lesions was measuring 7.0 × 6.6 × 6.0 cm with central cavitation in the right lower lobe (Fig. 3). Few enlarged lymph nodes were noted in the right upper para-tracheal, pre vascular, and sub-carinal regions. An irregular soft tissue density mass lesion was seen in the right breast measuring 2.2 × 3.7 cm. There was no infiltration into the skin and chest wall. Subsequently, a right breast ultrasound was done (Fig. 4) which revealed an irregular, ill-defined, hypoechoic mass lesion of size 2.0 × 3.0 cm with posterior acoustic shadowing in the lower inner quadrant of the right breast. The lesion was highly suspicious for malignancy and classified as U – BIRADS 4c for which a PET/CT scan and tissue biopsy were recommended.
The PET/CT scan (Fig. 5) revealed mild soft tissue thickening with increased metabolism in the posterolateral wall of the nasopharynx, nasal septum, and lingual tonsils and in the left carotid space near the skull base causing compression on the left recurrent laryngeal nerve and left vocal cord palsy. Additionally, multilobulated soft tissue density lesion with increased metabolic activity was seen in the right breast (SUV max- 4.96) along with hypermetabolic soft tissue density lesions in bilateral lungs, largest lesion (SUV max- 6.95) showing central cavitation in the right lower lobe. Furthermore, there was splenomegaly with diffuse ill-defined hypermetabolic nodular lesions (Fig. 6). Overall, the PET/CT imaging findings were suggestive of inflammatory/ granulomatous etiology. Follow-up image-guided biopsies were done from the lung and breast lesions.
CT-guided biopsy of the right lung lesion and tru-cut biopsy of the right breast lesion was done (Fig. 7)The histopathological examinations of the right lung and breast mass lesions confirmed necrotizing granulomatous inflammation (Fig. 8) and no evidence of malignancy. Furthermore, immunohistochemistry revealed elevated PR3-C ANCA (> 200 RU/ml). ANCA by immunofluorescence showed positivity for C-ANCA while P-ANCA and antinuclear antibodies were negative.
Hence, the patient was treated with 3 pulses of Methyl Prednisolone followed by 1 dose of IV cyclophosphamide. A follow-up breast ultrasound done after two months showed resolving mastitis (Fig. 9). However no follow-up CT was done to check for the resolution of the other lesions.