Malignant Neoplasms of the Colon
Philip H. Gordon, Santhat Nivatvongs, Lee E. Smith, Scott Thorn Barrows, Carla Gunn, Gregory Blew, David Ehlert, Craig Kiefer, Kim Martens in Neoplasms of the Colon, Rectum, and Anus, 2007
Primary plasmacytoma involving the colon is an exceedingly rare lesion (1079,1080). Presenting symptoms are nonspecific for gastrointestinal disease and may include abdominal pain, rectal bleeding, weight loss, nausea, vomiting, and anorexia. The lesion may be single or multiple and consists of polypoid or nodular protrusions. In the presence of intestinal involvement, appropriate scans and bone marrow biopsy should be obtained to rule out bone and marrow involvement. Microscopically, the plasmacytoma lesion is composed of many plasma cells. In most cases treatment has consisted of resection of the involved colon. An 80% 10-year survival can be expected (1037). However, if the diagnosis can be made by colonoscopic biopsy, treatment options include chemotherapy and radiotherapy (1081).
The Non-Hodgkin’s Lymphomas and Plasma Cell Dyscrasias
Harold R. Schumacher, William A. Rock, Sanford A. Stass in Handbook of Hematologic Pathology, 2019
Solitary plasmacytoma is a monoclonal plasma cell tumor that involves a single site. Patients suspected to have a solitary plasmacytoma must receive a complete evaluation to exclude plasma cell myeloma. Solitary plasmacytomas can arise in soft tissue, particularly in the head and neck (solitary extramedullary plasmacytoma), bone (solitary plasmacytoma of bone), or rarely lymph node. Up to one-half of patients with solitary plasmacytoma have an M-protein in serum (usually less than 2 g/dL) or urine, but other immunoglobulin levels are not decreased. There is no anemia, renal failure, or hypercalcemia. Solitary extramedullary plasmacytomas most commonly arise in the head and neck, particularly in mucous membranes of the upper respiratory tract, but may arise in other sites. Patients with solitary plasmacytoma of bone present with a single bone lesion, most often in a vertebra, the skull, or pelvis. They have no evidence of other bone lesions on radiographic studies, and random bone marrow biopsies fail to demonstrate plasma cell infiltrates.
Different Types of Leukemias, Lymphomas, and Myelomas
Tariq I Mughal, John M Goldman, Sabena T Mughal in Understanding Leukemias, Lymphomas, and Myelomas, 2017
This is a rare syndrome in which a serum paraprotein is associated with peripheral nerve function abnormalities of the sensorimotor variety (polyneuropathy—P), enlargement of organs, in particular the liver (organomegaly—O), hormonal disturbances (endocrinopathy—E), monoclonal gammopathy (M), and skin changes, in particular hyperpigmentation (S). Interestingly the disease occurs more frequently in Japan, where it is known as Crowe-Fukase syndrome (after Crowe, a British physician and Fukase, a Japanese physician). About 50% of cases are associated with myeloma and most of the remainder with a solitary plasmacytoma. The underlying mechanism for these multisystem changes remains unknown at present. It is also known as osteosclerotic myeloma and multicentric Castleman disease.
Vitamin B6 deficiency as a cause of polyneuropathy in POEMS syndrome: rapid recovery with supplementation in two cases
Published in Hematology, 2022
Hajime Yasuda, Yoshiki Furukawa, Kenya Nishioka, Makoto Sasaki, Yutaka Tsukune, Shuichi Shirane, Nobutaka Hattori, Miki Ando, Norio Komatsu
Case 2 is a 51-year-old man presenting with polyneuropathy involving all four limbs, edema, hyperpigmentation, IgG-lambda type M-protein, and an elevated serum VEGF of 3350 pg/mL. CT scans revealed a thoracic spine tumor (T2-4) that was compressing the spinal cord parenchyma, and multiple osteosclerotic and osteolytic lesions in the thoracic spine. The thoracic spine tumor was resected to relieve spinal cord compression, and pathology revealed a plasmacytoma. Thus, multiple myeloma complicated by POEMS syndrome was diagnosed. Postoperative radiation therapy of 24Gy was administered to the original site of plasmacytoma, and three courses of VRD (bortezomib, lenalidomide, dexamethasone) therapy were administered. Although the patient’s gait disturbance improved and was able to walk with a cane after resection of the plasmacytoma, thereafter, his symptoms became fixed for approximately six months and numbness and loss of sensation especially in the lower extremities persisted. In October 2020, VB6 levels were found to be low at 3.8 ng/mL, and supplementation with 60 mg/day of PPH was initiated (Figure 1). Subsequently, the patient’s lingering symptoms of polyneuropathy suddenly started to improve, and he became cane-free and his gait almost normalized within two months after initiation of VB6 supplementation. The patient underwent two more courses of VRD therapy and proceeded to ASCT with melphalan 200 mg/m2 conditioning in December 2020. The clinical course of case 2 has been previously reported elsewhere [7].
Multiple myeloma presenting as plasmacytoma causing obstructive jaundice
Published in Baylor University Medical Center Proceedings, 2020
Ted George Achufusi, Anuj Sharma, Bishnu Sapkota
A 72-year-old white man presented to our institution with complaints of abdominal discomfort and painless jaundice. On admission, his lab work was significant for abnormal liver biochemistries with alanine transaminase 417 IU/L, aspartate aminotransferase 414 IU/L, alkaline phosphatase 1309 IU/L, total bilirubin 15.9 mg/dL, and direct bilirubin 13.4 mg/dL, with normal lipase, amylase, and white blood cell count. Physical exam revealed diffuse jaundice; however, abdominal exam did not reveal any focal findings. Computed tomography of the abdomen and pelvis was significant for a large mass adjacent to the pancreatic head along with multiple retroperitoneal and intra-abdominal nodules (Figures 1 and 2). Subsequently, the patient underwent EUS-FNA of the pancreatic lesion. Tissue biopsy was positive for plasmacytoma. Histology showed sheet-like proliferation of predominantly immature atypical plasma cells with prominent nucleoli, with scattered mature plasma cells in the background.
Five cases of orbital extramedullary plasmacytoma: diagnosis and management of an aggressive malignancy
Published in Orbit, 2019
Samuel S. Y. Wang, Mitchell B. Lee, Adarsh George, Sarah B. Wang, Jonathan Blackwell, Steve Moran, Ian C. Francis
Reports of plasmacytoma involving the orbit are rare.8,10–13 These cases have included reports of plasmacytoma in the orbit, conjunctiva, and iris. Most of these cases are reported in the context of multiple myeloma, whilst the remainder consists of solitary plasmacytomas. In some cases, the presentation of orbital plasmacytoma preceded the diagnosis of multiple myeloma.14 In the current series, three out of five patients presented with secondary orbital plasmacytomas as the manifestation of a recurrence of their multiple myeloma. In Cases 2 and 3, the orbital plasmacytomas were features of the initial multiple myeloma presentation. These were not SEPs, as they presented in conjunction with evidence of systemic multiple myeloma.
Related Knowledge Centers
- Respiratory Tract
- Bone Marrow
- Axial Skeleton
- Neoplasm
- Plasma Cell
- Soft Tissue
- Multiple Myeloma
- Plasma Cell Dyscrasias
- Monoclonal Gammopathy
- Radiation Therapy