PMC6230411_01
(a) PET CT of 2011 revealed a soft tissue paravertebral mass extending from the T7 till L1. (b, c) Contrast-enhanced computed tomography (CT) scan showing para-aortic soft tissue thickening as well as retrocrural thickening. (d) Bilateral mild hydronephrosis.
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fig1
Radiographic workup revealed a soft tissue paravertebral mass extending from the T7 till L1 (Figure 1). In 2015 and 2016, repeated imaging showed disease progression and development of mild bilateral hydronephrosis (Figures 1-1).
Histological features of retroperitoneal mass in case 1. (a) Low-power magnification shows extensive sclerosis with mixed inflammatory infiltrate and occasional lymphoid aggregates. (b) High-power magnification shows obliterative phlebitis and numerous lymphocytes and plasma cells in the background.
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fig2
Due to presence of sclerosing fibrosis (Figure 2(a)) and obliterative phlebitis (Figure 2(b)), IgG4 immunostaining was performed, and it showed moderate numbers of IgG4 plasma cells with a IgG4/IgG plasma cell ratio of >40.
PMC6230411_02
(a, b) Contrast-enhanced CT scan neck showing lobulated enhancing lesion in the deep lobe of the left parotid gland about 2.3 x 2.0 cm. Similar enhancing soft tissue thickening is seen involving adjacent left carotid space reaching up to the base of the skull. (c, d) MRI neck and brain with FLAIR images showing the same lesion in the left parotid gland and carotid space. (e) MRI brain showing involvement of the left 12th cranial nerve at the skull base. (f, g) PET CT presteroid and rituximab (July 2016) and steroid and rituximab (November 2016) showing significant resolution. (h) Proton radiotherapy. (i, j) MRI brain done in August 2017 showing extensive diffuse irregular enhancing subependymal lesions (axial views). (k, l) MRI brain done in August 2017 showing extensive diffuse irregular enhancing subependymal lesions (sagittal views). (m, n) MRI brain done in October 2017 showing significant regression of all lesions after treatment with steroid and rituximab.
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fig3
After receiving high-dose steroids followed by rituximab therapy, she had significant improvement and repeated PET-CT after 4 weeks showed significant improvement (Figures 3 and 3). She was treated with proton therapy form March till May 2017 without significant improvement (Figure 3). MRI brain was performed on 29/8/2017 (Figures 3-3). Follow-up MRI brain was done on 5/10/2017 (Figures 3 and 3).
PMC6230411_03
PMC6230411_04
(a, b) MRI head and neck done in May 2013 showing diffuse lacrimal gland enlargement and features of sinusitis. (c) MRI head and neck done in May 2013 showing bilateral parotid and submandibular gland enlargement. (d, e) Follow-up MRI in August 2014 showing significant regression in pan sinusitis, regression in the lacrimal gland, and the salivary gland enlargement after treatment with steroid and azathioprine. (f, g) Follow-up MRI in February 2017 showing disease progression despite steroid and azathioprine.
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fig4
MRI and biopsy. MRI neck and orbits showed the following: bilateral lacrimal glands swelling and enhancement, bilateral parotid and submandibular glands enlargement, multiple intraparotid lymphadenopathy, cervical lymphadenopathy and features of sinusitis (Figures 4-4). Repeated MRI showed significant response to therapy (Figures 4 and 4). MRI head and neck was repeated on 8/2/2017 (Figures 4 and 4) and showed increase in enlargement of bilateral lacrimal glands, submandibular glands, parotid glands with intraparotid nodes, and cervical lymph nodes by size and numbers.
Histological examination of the submandibular gland in case 3. (a) There is dense lymphoplasmacytic infiltrate with acini atrophy and mild periductal fibrosis. No phlebitis seen. (b) IgG4 immunostain occasional clusters of positive plasma cells 10-20 per high-power field.
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It showed patchy dense lymphoplasmacytic infiltrate (Figure 5(a)) with occasional clusters of plasma cells. These plasma cells were mostly positive for IgG4 immunostain (Figure 5(b)) with 10-20 cells per high-power field.
PMC6230411_05
Histological examination of ampullary mass in case 4. (a) Histological examination revealed villotubular structures lined by dysplastic epithelium consistent with adenoma. (b) High-power examination revealed numerous clusters of plasma cells in the intervening lamina propria.
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The biopsy showed ampullary adenoma with high-grade dysplasia (Figures 6(a) and 6(b)).