POEMS syndrome with vascular transformation of the lymph node sinuses: A case report

Abstract

Abstract. POEMS syndrome is a rare multisystem disorder associated with the clinical signs of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. However, there is often a delay in the diagnosis due to a lack of overall consideration of the symptoms collectively. For this reason, POEMS syndrome is frequently mistaken for other diseases, such as chronic inflammatory demyelinating polyneuropathy. The present study reports the case of a 40-year-old female patient, who presented with a progressive lack of strength in the lower limbs and a unilateral cervical lump. The patient's enlarged cervical lymph nodes were mistaken for local hemangioma. However, subsequently POEMS syndrome with vascular transformation of the lymph node sinuses (VTS) was diagnosed. The patient received glucocorticoid treatment (20 mg prednisone acetate, daily), which is ongoing. The most recent follow-up examination revealed that the patient's strength had improved and at the time of writing the patient remained alive. The study discusses the clinical manifestations, auxiliary examinations and reason for the misdiagnosis. Hematoxylin and eosin staining and cluster of differentiation 31 immunostaining were adopted to identify the VTS. To the best of our knowledge, this is the first report of POEMS syndrome with VTS.

Nodular Spindle Cell Vascular Transformation in Pelvic Lymph Nodes With Discovered on GIST1 (DOG1) Positivity Mimicking Metastatic Gastrointestinal Stromal Tumor

“…VTS is often seen in combination with a neoplasm in its proximity, with renal cell carcinoma being the most commonly reported [3,4]. Several cases have been reported of VTS association with liver cirrhosis, metastatic gastric cancer, myelodysplastic syndrome, and polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, skin changes (POEMS) syndrome [4][5][6][7][8]. It is commonly located in the intra-abdominal lymph nodes as seen in our patient, but cervical lymph node vascular transformations have also been documented [9].…”