A baseline tracheotomy tube placement rate of 0.7% was observed in this single-site tertiary NICU setting. Decannulation was accomplished early in life in two thirds of the surviving infants. Those infants failing decannulation had either severe underlying pulmonary or neurologic disorders.
Castleman’s disease is an uncommon benign lymphoproliferative disorder that commonly involves the mediastinum. We report an unusual case that involves the presentation of unicentric Castleman’s disease in a 52-year-old female. The patient had a supraclavicular mass extending onto the brachial plexus. The approach to the treatment and plan for supraclavicular masses is complex due in part to the extensive list of differential diagnoses possible. In this case specifically, while the mass was ultimately determined to be benign, post-surgery, the location of the mass intraoperatively made for a very technically challenging and complex dissection. Post surgical resection, the patient reported no complications. This case highlights the importance of clinical judgement, imaging and surgical technique in removing a mass encroaching on the brachial plexus.
Top of Form Kaposi's Sarcoma (KS) and Multicentric Castleman's Disease (MCD) can infrequently occur simultaneously in immunocompromised patients. Here we present a rare case of HIV negative, HHV8 positive, EBV positive MCD with simultaneous pure nodal KS in an immunocompetent patient. A 69 year old female with no past medical history presented in June 2015 with neck pains, night sweats, and 20 pound weight loss. Physical examination revealed bilateral cervical, inguinal lymphadenopathy. She also had bulky bilateral axillary lymphadenopathy. No skin lesions were noted. Excisional lymph node axillary biopsy performed 7/16/15 revealed follicular hyperplasia with intense follicular expansion of plasma cells (figure 1, 2). Some follicles showed transformed germinal centers with expanded mantle zones and occasionally more than one germinal cell within a single mantle (figure 1, 2). By Immunohistochemical stains, CD20 highlighted the follicular B-cells and expanded mantle zone B-cells. CD3 highlighted small interfollicular T-cells. CD138 highlighted interfollicular expansion of plasma cells that were positive for kappa and lambda light chains in the interfollicular areas by in-situ hybridization. The plasmablastic cells in the mantle zone showed lambda light chain restriction and HHV-8 positivity. HHV-8 additionally highlighted the nuclei of the spindle cell proliferation (figure 3). PET CT 7/14/15 showed bilateral axillary adenopathy with SUV 8.0, mild hilar adenopathy with SUV 6.0 (Figure 4) and prominent adenopathy of pelvic sidewall and inguinal regions with SUV ranging 3.0-8.0. Baseline labs 7/14/15: HIV (-), HHV8 DNA (-), EBV IgG 7.7 AI (+), EBV DNA (+), IL-6 elevated 7.91 pg/mL and CRP 3.64 mg/dL (high). CBC and CMP were normal. Serum immunoglobulins: IgA 653m/dL (high), IgG 3210 mg/dL (high), IgM 33 mg/dL (low). SPEP with immunofixation showed hypergammaglobulinemia with slight peak asymmetry of the gamma globulins without evidence of monoclonality. LDH was normal at 127 U/L. The patient was given Rituximab 375m/m2 IV day 1 and Liposomal Doxorubicin 20 mg/m2 IV day 1 (R-Dox) every 3 weeks for 4 cycles between 8/7/15 - 10/5/15 with complete resolution of lymphadenopathy clinically and by PET CT 10/15/15. Patient has been doing well without evidence of recurrence as of last clinic visit 3/14/16. Figure 1 Low power (5X magnification) showing targetoid pattern of follicles and prominent interfollicular stroma with prominent capillaries consistent with Castleman's and a spindle cell proliferation towards the bottom consistent with Kaposi Sarcoma Figure 1. Low power (5X magnification) showing targetoid pattern of follicles and prominent interfollicular stroma with prominent capillaries consistent with Castleman's and a spindle cell proliferation towards the bottom consistent with Kaposi Sarcoma Figure 2 Higher magnification (10X) shows a prominent follicle with concentric layering of peripheral lymphocytes that resembles onion-skin. To the right, the KS shows spindle cells forming slits with extravasated red blood cells. Figure 2. Higher magnification (10X) shows a prominent follicle with concentric layering of peripheral lymphocytes that resembles onion-skin. To the right, the KS shows spindle cells forming slits with extravasated red blood cells. Figure 3 HHV-8 nuclear immunostain highlights KS cells with nuclear staining. Figure 3. HHV-8 nuclear immunostain highlights KS cells with nuclear staining. Disclosures No relevant conflicts of interest to declare.
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