Duodenal Ulcer with Massive Gastrointestinal Hemorrhage as an Initial Manifestation in Multiple Myeloma with Extramedullary Disease: A Case Report

Plasma cell neoplasms are characterized by dysregulated proliferation of mature B cells, which can present with either single (solitary plasmacytoma) or systemic (multiple myeloma (MM)) involvement. MM with extramedullary plasmacytoma (EMP) is a rare disease that accounts for approximately 3–5% of all plasmacytomas. EMP with gastrointestinal (GI) system involvement is an even rarer entity, accounting for <1% of MM cases. We present a case of aggressive MM with EMP invading the duodenum, initially presented with massive upper GI hemorrhage and small bowel obstruction. A 67-year-old woman was admitted to our hospital owing to a lack of either gas or feces passage for 3 days. Abdominal distention and vomit with a high coffee ground content were observed for 24 h. The patient’s condition was initially diagnosed as small bowel obstruction, upper gastrointestinal bleeding, severe anemia, acute renal failure, and hypercalcemia. Furthermore, an analysis of immunoelectrophoresis in the blood, bone marrow aspiration, and tissue biopsy supported the diagnosis of MM and EMP invading the duodenum, upper GI hemorrhage, and small bowel obstruction. Our study provided the possible involvement of MM and EMP in the differential diagnosis of patients with unexplained GI hemorrhage and small bowel obstruction. A thorough review of the literature regarding the association between MM, GI hemorrhage, and small bowel obstruction is presented in this study.

Inositol Polyphosphate 4-Phosphatase Type II Is a Tumor Suppressor in Multiple Myeloma

Inositol polyphosphate-4-phosphatase type II (INPP4B) has been identified as a tumor suppressor, while little is known about its expression and function in multiple myeloma (MM). In this study, we evaluated the expression of INPP4B in 28 cases of newly diagnosed MM patients and 42 cases of extramedullary plasmacytoma (EMP) patients compared with normal plasma cells and found that low INPP4B expression was correlated with poor outcomes in MM patients. Moreover, expression of INPP4B in seven MM cell lines was all lower than that in normal plasma cells. In addition, loss of function of INPP4B promoted cell proliferation in MM cells; however, gain of function suppressed MM cells proliferation and arrested the cell cycle at G0/G1 phage. Meanwhile, knockdown of INPP4B enhanced resistance, but overexpression promoted sensitivity to bortezomib treatment in MM cells. Mechanistically, we found that INPP4B exerted its role via inhibiting the phosphorylation of Akt at lysine 473 but not threonine 308, which attenuated the activation of the PI3K/Akt/mammalian target of rapamycin (mTOR) signaling pathway. Therefore, we identified an inhibitory effect of INPP4B in MM, and our findings suggested that loss of INPP4B expression is a risk factor of aggressive MM.

Single-Cell Transcriptomes Combining with Consecutive Genomics Reveal Clonal Evolution and Gene Regulatory Networks in Relapsed and Refractory Multiple Myeloma

This study attempted to investigate how clonal structure evolves, along with potential regulatory networks, as a result of multiline therapies in relapsed/refractory multiple myeloma (RRMM). Eight whole exome sequencing (WES) and one single cell RNA sequencing (scRNA-seq) were performed in order to assess dynamic genomic changes in temporal consecutive samples of one RRMM patient from the time of diagnosis to death (about 37 months). The 63-year-old female patient who suffered from MM (P1) had disease progression (PD) nine times from July 2017 [newly diagnosed (ND)] to Aug 2020 (death), and the force to drive branching-pattern evolution of malignant PCs was found to be sustained. The mutant-allele tumor heterogeneity (MATH) and tumor mutation burden (TMB) initially exhibited a downward trend, which was then upward throughout the course of the disease. Various somatic single nucleotide variants (SNVs) that had disappeared after the previous treatment were observed to reappear in later stages. Chromosomal instability (CIN) and homologous recombination deficiency (HRD) scores were observed to be increased during periods of all progression, especially in the period of extramedullary plasmacytoma. Finally, in combination with WES and scRNA-seq of P1-PD9 (the nineth PD), the intro-heterogeneity and gene regulatory networks of MM cells were deciphered. As verified by the overall survival of MM patients in the MMRF CoMMpass and GSE24080 datasets, RUNX3 was identified as a potential driver for RRMM.

Parotid Salivary Gland Extramedullary Plasmacytoma with Local Lymph Node Metastasis in a Dog

ABSTRACT A 12 yr old spayed female mixed-breed dog presented for evaluation of a recurrent soft-tissue sarcoma. On physical examination, a firm mass was palpated ventral to the left ramus of the mandible. A fine-needle aspirate of the mass was suggestive of a round-cell neoplasm. A complete blood count, serum biochemical profile, and an abdominal ultrasound with liver and splenic aspirates were performed, and no clinically relevant abnormalities were identified. Advanced imaging of the skull identified an enlarged parotid salivary gland and an enlarged ipsilateral medial retropharyngeal lymph node. The medial retropharyngeal lymph node was sampled via fine-needle aspiration, and a round-cell population similar to what was present in the mass was identified. An incisional biopsy was performed under general anesthesia, which yielded a diagnosis of salivary gland extramedullary plasmacytoma, confirmed with immunohistochemistry (MUM-1). The parotid salivary gland and medial retropharyngeal lymph node were then surgically excised, and metastasis to the lymph node was confirmed by histopathology. The dog remained alive for 685 days after surgery until she was euthanized for hindlimb paresis of undetermined cause.

A novel differential diagnosis for diffuse cavitating nodules: primary pulmonary plasmacytoma

Background Extramedullary plasmacytoma is a rare monoclonal plasma cell neoplasm that originates from tissues other than the bone marrow and constitutes only 3%–5% of all plasma cell neoplasms. Most cases involve the upper respiratory tract. Extramedullary plasmacytoma involving the lung is extremely rare. Primary pulmonary plasmacytoma is a rare type of extramedullary plasmacytoma, usually presenting with a nodule or mass in hilar region. Literature search has shown very few cases of immunohistochemically confirmed cases of pulmonary plasmacytoma. Diffuse pulmonary infiltration is an unusual presentation. Case presentation We report the case of a 56 year old lady with history of cough and breathlessness since one month. Computed Tomography revealed diffusely scattered multiple cavitating nodules and consolidation in both lungs. Computed Tomography guided biopsy of one of the lung nodules was done. Histopathologic examination and immunohistochemistry showed features of pulmonary plasmacytoma .This is an unique case of primary pulmonary plasmacytoma with the rare presentation as diffusely scattered multiple cavitating nodules and consolidation. According to our literature search, primary pulmonary plasmacytoma manifesting as cavitating nodules is being reported for the first time. Conclusions Primary pulmonary plasmacytoma should be also be considered in the differential diagnosis of multiple diffusely scattered cavitating lung nodules.

Refractory primary extramedullary plasmacytoma in kidney: a case report

Extramedullary plasmacytoma (EMP) is a rare plasma cell neoplasm, with the majority (80–90% of cases) occurring in the upper aerodigestive tract. To our best knowledge, primary EMP from renal tissues is extremely rare. Herein, the diagnosis and treatment of a refractory primary EMP with renal involvement in a 53-year-old male patient is reported. The patient received radical nephrectomy followed by radiotherapy, and showed relapse 3 months after treatment. The cancer cells were sensitive to subsequent chemotherapy, however, the patient died of infection associated with the disease after almost 3.5 years following first presentation.

Extramedullary plasmacytoma of the nasal inferior turbinate: a case report

An extramedullary plasmacytoma (EMP) is a rare neoplasm characterized by monoclonal proliferation of plasma cells without features of multiple myeloma. Most EMPs occur in the head and neck region, especially in the aerodigestive tract. We herein report a case of an EMP arising from the nasal inferior turbinate. The mass was surgically removed, and a short cycle of radiotherapy was performed after the surgery. There was no recurrence of the tumor after 1 year of follow-up. These result may be useful for physicians who encounter similar situations in clinical practice.

Extramedullary Plasmacytoma of the Sphenoid Sinus presenting with visual loss: a Case Report

A rare case of sphenoid plasmacytoma in a 57-year old female who was presented with a frozen eye is reported. It was histopathologically confirmed based on transsphenoidal biopsy. Surgical removal of the tumor with complementary radiotherapy was performed as a treatment modality