Plasmacytoma Presenting Research Articles

Atypical Presentation of Plasmacytoma in a Young Woman

Atypical Presentation of Plasmacytoma in a Young Woman

A rare case of solitary plasmacytoma presenting as a skull lesion

A rare case of solitary plasmacytoma presenting as a skull lesion

A case report on pancreatic plasmacytoma presenting as obstructive jaundice

Background: Extramedullary solitary plasmacytomas (EMD) are an uncommon manifestation of plasma cell disorders. The median survival rate is less than 6 months. EMD rarely involves the digestive tract, the liver is the most common site with only a 2.3% incidence rate for pancreatic involvement. Case Presentation: We report a case of a patient with a history of multiple myeloma who presented with painless obstructive jaundice and EUS findings of hypoechoic, solid irregular mass in the pancreatic head with surrounding peripancreatic lymphadenopathy. Fine needle aspirate (FNA) confirmed malignant infiltration of plasma cell neoplasm. The patient rapidly deteriorated and succumbed to his illness within 6 weeks of diagnosis. Conclusion: Extramedullary plasmacytomas (EMD) involving the pancreas are uncommon and associated with a poor prognosis. Our case highlights the importance of considering extramedullary plasmacytoma when a patient with MM presents with a pancreatic mass, particularly one showing a rapid interval growth. This rate of growth is not seen with primary pancreatic parenchymal diseases such as cancer, cysts, and so on. This case highlights the importance of considering rare differentials while evaluating patients with pancreatic mass.

Localised plasmacytoma presenting as right chest wall swelling

Development of any swelling in the right lower chest wall or right upper abdomen is an unusual presentation. Hence the localisation of the origin of swelling becomes difficult to ascertain through clinical examination alone. Accurate diagnosis requires detailed investigation and ruling out of differentials one by one. We would like to share one such case of a patient presenting to surgery OPD with complaint of swelling in the lateral aspect of right chest wall which on investigations turned out to be a rib tumour arising from the bone marrow. Lady of age 57 years presented to us with h/o swelling in lateral aspect of right chest wall which gradually increased in size over 3 years and associated with pain for last 5 months. On exam an oval swelling of size 8×5 cm seen in lateral wall of right lower chest wall. On detailed investigations, the swelling was diagnosed as a plasma cell tumour of right 8th rib. Any chest wall or abdominal swelling needs detailed evaluation before proceeding for surgical intervention. It is important to keep various differential diagnosis in mind considering clinical factors and site of swelling to make an accurate diagnosis.

S4083 A Rare Case of Duodenal Plasmacytoma Presenting as Abdominal Pain and Jaundice

S4083 A Rare Case of Duodenal Plasmacytoma Presenting as Abdominal Pain and Jaundice

S1927 Pancreatic Plasmacytoma Presenting as Obstructive Jaundice

S1927 Pancreatic Plasmacytoma Presenting as Obstructive Jaundice

Extramedullary plasmacytoma presenting as a rapidly growing lateral neck mass: A case report

Introduction: We detail the rare clinical presentation of an extramedullary plasmacytoma as a rapidly growing lateral neck mass. The pathology and its management are discussed. A rapidly growing extramedullary plasmacytoma infiltrating the cervical spine. Case Report: A 53-year-old male presented to our emergency room with a one-month history of worsening left-sided neck pain and an enlarging lateral neck mass. Evaluation with cervical imaging and laboratory analysis were performed. The patient underwent a soft tissue biopsy with subsequent spinal cord decompression and an occipital-cervical fusion. Pathology revealed the diagnosis of plasmacytoma. The patient was referred to oncology and radiation therapy for adjuvant treatment. Conclusion: This case represents the unusual presentation of a rapidly growing extramedullary plasmacytoma to the soft tissues of the neck with subsequent erosion of the base of the skull and upper cervical vertebral spine. It was treated effectively with posterior cervical decompression, arthrodesis, and adjuvant radiation and chemotherapy.

Progression of Multiple Myeloma Presenting as Subdural Plasmacytoma (P9-13.006)

<h3>Objective:</h3> N/A <h3>Background:</h3> The American Cancer Society estimates that one in 130 adults will be diagnosed with multiple myeloma (MM) at some point in their lifetime. Extramedullary plasmacytoma is a rare manifestation of multiple myeloma. Intracranial, extramedullary plasmacytoma is an extremely rare presentation of this condition that can lead to diagnostic confusion and may be confused to solid neoplasm metastasis, hemangiopericytoma, or even a subdural hematoma depending on the lesion’s location. <h3>Design/Methods:</h3> N/A <h3>Results:</h3> We report the case of a 79-year-old female with known MM who presented with four months of slowly progressive gait instability, falls, and dizziness. On presentation, the patient was found to have a non-focal neurologic exam and initial CT head revealed a 1.2 cm subdural collection with 1.4 cm of midline shift and associated sulcal effacement that was felt to most likely represent subdural mass vs. subdural hematoma. She was taken to the operating room where a subdural mass was encountered and intraoperative pathology was consistent with plasmacytoma. The patient underwent radiation therapy post-operatively, and was noted to be doing well until being found with decreased consciousness after three radiation treatments. CT head demonstrated severe worsening of cerebral edema for which she was transferred to the Neuro ICU and ultimately treated with hyperosmolar therapy until her mental status improved. She progressed well following this and was discharged to post-acute rehabilitation. <h3>Conclusions:</h3> This case highlights a rare presentation of extramedullary plasmacytoma of the subdural space. A broad differential and high index of suspicion for neoplastic process were key in establishing the patient’s diagnosis in our case and helped to inform the treatment approach, which consisted of biopsy followed by radiation therapy. In addition, our case highlights the potential complications that can arise with radiation therapy for subdural plasmacytoma, namely the large degree of edema that was encountered after relatively few radiation treatments. <b>Disclosure:</b> Dr. West has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Surgical Neurology International. Dr. Mason has nothing to disclose.

A case of incidental multiple Myeloma presenting with pathologically proven large frontal skull plasmacytoma and review of therapeutic targets: Case report

Introduction: Multiple myeloma and plasmacytoma are both plasma cell malignancies that belong to the family of blood dyscrasias. They are thought to be different entities but can occur simultaneously in some patients. Case Report: We present a unique case of a large plasmacytoma heralding the diagnosis of multiple myeloma with a preceding history of trauma which makes this case peculiar as this is an important addition to the limited existing scientific literature. The index patient was largely asymptomatic despite having a massive frontal skull/lobe lesion measuring approximately 8 × 6 cm in size alongside a chest wall mass involving the anterior left 6th rib, and a mildly displaced acute pathological fracture of the left clavicle. A primary diagnosis of multiple myeloma was made following serum protein electrophoresis with pathological confirmation of frontal skull plasmacytoma. After multidisciplinary discussion, radiotherapy was administered for plasmacytoma with complete resolution of the frontal skull mass without the need for resection and chemotherapy for multiple myeloma with daratumumab in combination with lenalidomide-dexamethasone—chosen due to anticipated poor outcome at the time of diagnosis. Autologous stem cell transplantation was subsequently instituted for multiple myeloma with a good outcome to date. Conclusion: Solitary plasmacytoma can co-exist with multiple myeloma and the prognosis remains poor in such circumstance. Patients should be followed up closely with yearly imaging—magnetic resonance imaging (MRI)/computed tomography (CT), and positron emission tomography (PET) scans. A pivotal lesson from this case report emphasizes the atypical presentation of plasmacytoma and multiple myeloma with the peculiarity of a prior history of trauma which is believed to be associated with plasma dyscrasias. Over the last decade, novel therapeutic agents such as chimeric antigen receptor T-cell therapy, which is the first approved cell-based therapy for multiple myeloma, antibody-drug conjugates, and a dozen of bi-specific antibodies/immunotherapy have been developed and shows promising outcome in the future, especially for refractory cases of multiple myeloma.

An unusual presentation of a secondary extramedullary plasmacytoma

Plasma cell dyscrasias are a group of entities characterized by neoplastic proliferation of a single clone of plasma cells, typically producing a monoclonal Immunoglobulin [1].

Progressive lower extremity weakness as the initial presentation of isolated large thoracic plasmacytoma

Plasmacytoma is a tumor of monoclonal plasma cells of bone or soft tissue that can occur anywhere in the body without evidence of systemic multiple myeloma. It may present as solitary or multiple masses and is classified as osseous if arising from bone or extraosseous if arising from soft tissue. Osseous plasmacytoma is the most common form of plasmacytoma with predominance in active hematopoietic bones, including vertebrae, femur, pelvis, and ribs. The diagnosis is made with a tissue biopsy. Different imaging modalities allow for tumor localization, and magnetic resonance imaging (MRI) is the gold standard to detect spinal cord compression. Corticosteroids provide analgesia, reduce vasogenic edema, and have anti-myeloma activity which may result in better neurological outcomes in cases of acute spinal cord compression. Corticosteroids should be started promptly once cord compression is suspected. We report a case of progressive lower extremity weakness as the initial presentation of a thoracic plasmacytoma. The patient was started on a high-dose corticosteroid after acute cord compression was suspected. Magnetic resonance imaging confirmed cord compression. T1-T2 corpectomy with C5-T5 posterior spinal fusion for decompression and stabilization was done. He was successfully discharged to an inpatient rehabilitation facility with plans for definitive radiotherapy. Worsening back pain and lower extremity weakness in elderly patients should raise concerns for acute cord compression. Early intervention to relieve compression is crucial to preserve neurological functions.&#x0D; &#x0D; Keywords: plasmacytoma, weakness, steroids, multiple myeloma

S2691 A Rare Presentation of Gastric Plastocytomas in a Patient With Multiple Myeloma

Introduction: Multiple myeloma is an uncommon hematological neoplasm. This malignancy involves unchecked plasma cell growth in the bone marrow, but in a subset of often more advanced cases, these cells may migrate to soft tissues causing extramedullary plasmacytoma. This is unusual, and, in the literature, few cases of extramedullary plasmacytomas involve the gastrointestinal tract. Within this system, the more common locations are the stomach, the liver, the large bowel, and more rarely, the small bowel. Case Description/Methods: A 59-year-old male with a medical history of chronic hepatitis C, and Stage III multiple myeloma status post four cycles of chemotherapy presented with altered mental status. Upon evaluation, he had significant electrolyte abnormalities, remarkable anemia, severe renal and liver dysfunction, and a positive hemoccult test. Our patient received intravenous fluid, and multiple blood products. He was then admitted to the intensive care unit for a high level of care. On the second day of admission, he developed new onset hematemesis, and the gastroenterology team was consulted. The patient was started on a pantoprazole drip. Urgent esophagogastroduodenoscopy showed several polypoid appearing masses with overlying ulceration in the gastric fundus (Figure a &b), and tissue biopsies were obtained. Biopsy result of the polypoid fundal mass showed a large cell neoplasm with plasmocytic differentiation. Gastric tissue sections showed an ulcerated lesion with submucosal, tightly packed atypical large cells with multinucleation, prominent nucleoli and relatively identifiable mitoses (Figurec). A panel of immunohistochemical stains with plasma cell markers were positive for kappa, Multiple Myloma-1 nuclear protein, and CD138 (Figure d-f). The overall findings were compatible with gastric involvement by a plasma cell neoplasm. Discussion: This case highlights a complicated, yet a uniquely rare presentation of extramedullary plasmacytoma in a patient diagnosed with multiple myeloma. Although the gastrointestinal tract is rarely involved in patients with multiple myeloma, it should be considered as the differential diagnosis in patients with gastrointestinal bleeding especially with a known concurrent systemic hematological malignancy. The presence of gastrointestinal involvement and the malignant phase of multiple myeloma are associated with a poor prognosis despite aggressive therapy, and it is therefore paramount to establish an accurate diagnosis early to avoid any delays in treatment.Figure 1.: (a,b) EGD shows polypoid EMP in the gastric fundus. (c) Infiltration of plasmablasts with highly atypical nuclei and prominent nucleoli (hematoxylin-eosin 20x). (d) Granular positivity for kappa immunoglobulin light chain. (e) Positive immunostaining for MUM-1. (f) Positive CD138 immunostaining highlights the plasma cells in the lamina propria of the gastric fundus EMP.

S1801 Pancreatic Plasmacytoma Presenting as Jaundice in Patient With Relapsed Multiple Myeloma

S1801 Pancreatic Plasmacytoma Presenting as Jaundice in Patient With Relapsed Multiple Myeloma

Thymic plasmacytoma presenting as polyneuropathy and revealing multiple myeloma: a case report

BackgroundMultiple myeloma (MM) is the most frequent malignant plasma cell disorder with proliferation of neoplastic plasma cells in the bone marrow or other tissue, most commonly in the upper aerodigestive tract. The invasion of the thymus is exceptional. Neurological complications are usual, but represent exceptionally the revealing symptom.Case presentationWe report a case of polyneuropathy revealing a thymic plasmacytoma as a mediastinal invasion of MM in a 48-year-old woman. She was admitted after developing progressive ascending distal paresthesias and weakness in lower limbs. Examination showed symmetrical distal sensorimotor impairment with axillary and inguinal adenopathies. Electroneuromyography revealed a sensorimotor length-dependent neuropathy. Serum protein electrophoresis showed monoclonal protein peak in β-γ globulin region. Immunoelectrophoresis showed IgA lambda monoclonal gammapathy. Myelogram and bone marrow biopsy revealed plasmocytosis of 5%. Chest computed tomography showed a histologically confirmed thymic plasmacytoma associated with a lytic lesion of the 5th rib leading to the diagnosis of MM.ConclusionsThe association between a thymic plasmacytoma and peripheral neuropathy is rare and a workup for MM is necessary to guide therapeutic management.

SOLITARY PRIMARY PULMONARY PLASMACYTOMA: A RARE PRESENTATION

TYPE: Abstract TOPIC: Lung Cancer INTRODUCTION: Solitary Primary Pulmonary Plasmacytomas (SPPP) is a rare presentation of solitary intramedullary plasmacytoma. We report a case of Solitary Primary Pulmonary Plasmacytomas with the absence of circulating immunoglobulins which is an extremely rare presentation. CASE PRESENTATION: A 80 year old asymptomatic non-smoker gentleman was evaluated for anemia. Work up for anemia including bone marrow, serum electrophoresis and urine bence jones protein was negative. The whole body PET-CT revealed a 3 x 2 cm solitary lung nodule with increased FDG uptake in the apical segment of the right lower lobe lung. CT guided biopsy was inconclusive. Transbronchial lung biopsy revealed malignant plasma cells with CD138, CD56, V-38 and lambda light chain restrictions. He was diagnosed with Solitary Primary Pulmonary Plasmacytomas. He underwent 25 fractions of 45 Gys radiation with complete resolution. On follow up after 1 year there was no clinical, radiological or biochemical evidence or further extramedullary plasmacytoma. DISCUSSION: Solitary Primary Pulmonary Plasmacytoma is a very rare manifestation of extramedullary plasmacytoma. Diagnosis is by transbronchial lung biopsy, CT guided or ultrasound-guided biopsy and rarely surgical lung biopsy. Treatment options include radiotherapy, surgery and chemotherapy. In select few radiotherapy alone may show an excellent response. CONCLUSIONS: Solitary Primary Pulmonary Plasmacytomas is a very rare condition with a non-specific presentation. A differential of Solitary Primary Pulmonary Plasmacytomas should be considered in patients with solitary hilar lesion with anemia. Radiotherapy alone can have a complete resolution. DISCLOSURE: Nothing to declare. KEYWORD: primary pulmonary plasmacytoma, rare presentation, solitary extramedullary plasmacytoma, Lung cancer

In correspondence to "Solitary extramedullary plasmacytoma presenting as an adrenal tumor: case report and literature review".

In correspondence to "Solitary extramedullary plasmacytoma presenting as an adrenal tumor: case report and literature review".

Solitary extramedullary plasmacytoma presenting as an adrenal tumor: case report and literature review

Solitary extramedullary plasmacytoma, an extremely rare case which accounts for about 3% of all plasma cell neoplasms, often occurs in the head and neck region such as para nasal sinuses, nasal cavity and oral cavity, it also occurs in the lymph nodes, lungs, thyroid, intestine, liver, pancreas, testis, breast, or skin. Isolated primary plasmacytoma of adrenal is extremely rare and only nine documented cases have been reported in the literature. We are presenting the 10th case which is the youngest patient until now with symptoms of fever and hepatosplenomegaly. A 19-year-old female was admitted with an irregular fever for 20 days. After a series of investigations were carried out there were no CRAB symptoms (hypercalcemia, renal failure, anemia and bone lesions), no free light chain and no more than 10% increase in plasma cell on bone marrow examination. Computed tomography (CT) scan revealed a tumor in the left adrenal region, and it was diagnosed to be a solitary extramedullary plasmacytoma on biopsy. She underwent the tumor resection one month after admission and recovered well after operation without fever and was discharged from hospital on the thirteenth post-operative day. She has been followed up for 5 years without any sign and symptom of tumor recurrence. Extramedullary plasmacytoma of adrenal gland in an extremely rare disease and usually diagnosed late in life but it can present in younger patients with variable symptoms. However, the surgical treatment yields excellent long-term results. So, complete surgical resection of the lesion is not only a good diagnostic measure, but also an intent-for-cure treatment for solitary adrenal extramedullary plasmacytoma.

Intraocular solitary extramedullary plasmacytoma presenting as unilateral anterior and intermediate uveitis preceded by refractory glaucoma

BackgroundSolitary extramedullary plasmacytoma (SEP) is a localised proliferation of monoclonal plasma cells involving soft tissue with no or minimal bone marrow involvement and no other systemic evidence of multiple myeloma. Intraocular involvement is exceedingly rare.Case presentationWe report a 78-year-old man who was referred with glaucoma in the right eye. He subsequently developed anterior chamber (AC) inflammation and refractory glaucoma then dense vitritis. A vitrectomy was performed with the biopsy revealing numerous plasma cells with atypical findings. In conjunction with the flow cytometry results, and a systemic work up excluding multiple myeloma, a diagnosis of SEP was made. The patient was treated with ocular external beam radiotherapy with resolution of the intraocular inflammation and control of the intraocular pressure. He remains well with no local recurrence and no development of multiple myeloma over a follow up period of 2.5 years.ConclusionsThis is the first case report of SEP presenting as intraocular inflammation without a uveal tract mass.

S2095 Gastric Extramedullary Plasmacytoma Presenting as a Solitary Gastric Mass

INTRODUCTION: Extramedullary plasmacytomas (EMP) are a subcategory of plasma cell neoplasm that involves organs outside the bone marrow. We report a case of an aggressive extramedullary myeloma invading the stomach. CASE DESCRIPTION/METHODS: An 84 year old lady with PMHx of DM, hypothyroidism, HTN and GERD presented to the ED with abdominal pain, nausea and vomiting for 2 days. Patient was found to be anemic with Hgb of 10.7 g/dL, hematocrit 32.4%. Patient’s electrolytes and renal function tests were within normal limits. Imaging (US) of the abdomen showed gallbladder calculus with positive murphy’s sign and no pericholecystic fluid. A CT scan of the abdomen and pelvis showed a large hiatal hernia with suspicion of cholelithiasis. A HIDA scan showed no evidence of cystic duct obstruction or distal biliary obstruction. After cholecystitis was ruled out GI was consulted and a EGD was planned for intractable abdominal pain with intermittent vomiting. During the EGD findings [Figure 1] were noted for a 4 cm non-bleeding, non-ulcerated mass on a very large stalk that appeared to be blocking the pylorus. The pylorus was normal appearing once past the mass. The polyp was partially resected. A small polyp was resected but the larger polyp was not able to be removed due to getting stuck at the cricopharyngeus so it remained in the stomach. Biopsy findings were noted predominantly of well-differentiated plasma cells with some of the cells extending to the mucosa. The immunohistochemical stains were positive for CD138, CD 79 [Figure 2] and showed a low Ki-67 proliferation index of < 5%. The neoplastic cells were lambda light chain restricted [Figure 3]. Rare plasma cells are positive for kappa. They were negative for PAX5, CD20, CD56, c-KIT, cyclin D1 and pankeratin. CD3 highlights background T-cells. Based on the findings a plasmacytoma or secondary involvement by multiple myeloma was favored. DISCUSSION: Plasmacytoma can be primary or secondary with secondary form more common. The most commonly involved sites are the liver, spleen, and lymph nodes. The prevalence of GI system involvement was found to be 0.9% in a recent systematic review done with 2,584 Multiple myeloma (MM) patients. This is associated with a poor survival of less than seven months from diagnosis. [1] Favorable outcomes in cases of EMP have been linked to tumor size < 4 cm, age < 50 years, patients with head and neck EMP, and serum M protein negativity. [2]. We have described a rare case of gastric plasmacytoma without symptoms of MM.Figure 1.: EGD finding of a mass obstructing the pylorus, injected with epinephrine and clamped before excision.Figure 2.: Cells stained with CD79A.Figure 3.: Monoclonal population lambda +.

Clinical presentation and outcomes of solitary plasmacytoma in a tertiary hospital in the UK

Solitary plasmacytoma is a rare localised neoplasm of monoclonal plasma cells. The standard treatment involves radical radiotherapy; however, a significant proportion of patients subsequently develop multiple myeloma. In this study, we evaluate the outcomes of solitary plasmacytoma in a retrospective cohort of patients treated in a single tertiary centre.The case records of plasmacytoma patients treated in a 15-year period were analysed and retrospectively followed up from the date of diagnosis. Thirty-four cases met the inclusion criteria; 27 (79%) solitary plasmacytoma of bone (SBP) and 7 (21%) extramedullary plasmacytoma (EMP). The thoracic vertebrae were the commonest sites for SBP while EMP occurred most frequently in the upper airway. Pain and spinal cord compression were the most frequent symptoms. A paraprotein was detectable in 18 (53%) patients. Over a median follow-up of 48 months, 13 (38%) developed multiple myeloma. The 5- and 10-year survival rates were 80% and 56%, respectively; median progression-free survival was 77 months. Four patients (12%) developed a second malignancy.Progression to multiple myeloma remains a formidable challenge in the management of solitary plasmacytoma, hence adjunct therapies are needed.