Secondary gastroduodenal plasmacytoma in a patient with multiple myeloma.

We present an exceptional case of secondary gastroduodenal plasmacytoma in the course of multiple myeloma and we discuss the clinical presentation, images obtained by endoscopy and computed tomography, treatment and clinical course.

Secondary Skin Plasmacytomas.

A 78-year old man was diagnosed in 2006 with IgAκ multiple myeloma (MM) (stage III-A). The patient was referred to our dermatology department in 2012 for evaluation of erythematous skin nodules on the anterior right aspect of the thorax; the skin lesions were noted during hospitalization for multiple bone fractures. He was on fourth-line chemotherapy (with vincristine/adriamycin/dexamethasone) because of constant disease progression. The patient was unaware of the skin lesions' evolution over time and did not recall when they had first appeared. He had no pain, itching, or spontaneous bleeding.

Metastatic Intracerebral Plasmacytoma Treated with Radiation and Thalidomide, Dexamethasone with Cyclophosphamide Chemotherapy.

Intracerebral plasmacytoma is an extremely rare disease for which no treatment protocol has been established. The authors present a case of metastatic intracerebral plasmacytoma that was partially resected and treated with radiation therapy. For tumor recurrence, a combination chemotherapy regimen was initiated. A 48-year-old male patient presented with dizziness and memory loss. Conventional magnetic resonance imaging (MRI) of the brain revealed a multilobulated mass with enhancing cystic and solid components measuring 7 × 7 × 6 cm in the left frontal lobe. The patient had undergone subtotal gastrectomy and transverse colectomy 8 years before admission and had been diagnosed with extraosseous plasmacytoma. At the time of the current presentation, the patient underwent craniotomy for the parenchymal lesion. Partial tumor resection was performed. Histologic and immunohistochemical examinations confirmed the diagnosis of plasmacytoma. Fractionated radiotherapy was administered, and no enhancing mass was observed on follow-up MRI. One year after radiotherapy, tumor recurrence was observed in a different area of the cerebral parenchyma. Combination thalidomide, dexamethasone, and cyclophosphamide chemotherapy was administered. After three cycles of chemotherapy, the tumor was well controlled on MRI. Hereafter, two more times of tumor recurrence occurred in the other sites of the cerebral parenchyma, but with chemoradiation therapy, the tumor was well suppressed. The findings of this case suggest that the cerebral parenchyma can be one of the metastatic sites for extraosseous plasmacytoma. In addition, combination chemotherapy with thalidomide, dexamethasone, and cyclophosphamide may be a useful treatment option for intracerebral plasmacytoma.

Anorectal involvement in a patient with multiple myeloma.

Multiple myeloma is a neoplastic proliferation of monoclonal plasma cells. Symptomatic gastrointestinal involvement is uncommon. We report the case of a 45-year-old patient admitted with an anorectal polypoid lesion, which progressed to colonic obstruction. Investigation revealed a secondary plasmacytoma associated with multiple myeloma. We discuss the characteristics of this rare entity with poor prognosis, its clinical implications and treatment options.

Cutaneous localization in multiple myeloma in the context of bortezomib-based treatment: how do myeloma cells escape from the bone marrow to the skin?

The skin is a possible site of extramedullary localization in multiple myeloma (MM) patients; however, the mechanisms involved in this process are poorly understood. We describe the case of a refractory MM patient who developed a cutaneous localization under bortezomib treatment and we further expanded observations in other eight MM patients. We focused on the expression of genes involved in plasma cell skin homing, including CCR10, which was highly expressed. Moreover, we observed a lack of CXCR4 surface expression and the down-regulation of ICAM1/CD54 throughout the progression of the disease, suggesting a possible mechanism driving the escape of MM cells from the bone marrow into the skin.

[Secondary cutaneous plasmacytoma revealing multiple myeloma: about a case]. / Plasmocytome cutané secondaire révélant un myélome multiple: à propos d'un cas.

Secondary metastatic cutaneous plasmacytoma is a multiple extramedullary plasma cell proliferation involving skin. Its diagnosis is based on the identification of malignant plasma cells proliferation in the bone marrow and in the skin. Its occurrence is associated with advanced myeloma and a poor prognosis.

Cutaneous manifestations of multiple myeloma and other plasma cell proliferative disorders.

Plasma cell proliferative disorders cause rare but extremely varied dermatologic manifestations that may occur as an accompaniment to established diagnoses, or may be a first clue of an underlying neoplasm in the setting of clinical suspicion. In some instances skin lesions result from aggregation of misfolded monoclonal immunoglobulins or their fragments, as in light chain-related systemic amyloidosis. On other occasions the cutaneous lesions result from deposits of malignant plasma cells or monoclonal proteins. In still others, the dermatologic manifestations are related to antibody activity of monoclonal protein, as in many cases of cryoglobulinemia. This report provides insights into the well-recognized cutaneous manifestations associated with plasma cell disorders.

Cutaneous and pleural involvement in a patient with multiple myeloma.

Multiple myeloma (MM) is a malignant proliferation of a single clone of plasma cells and an excess of monoclonal immunoglobulin production. It is rarely associated with cutaneous and pleural involvement. We report a new case of a 62-year-old woman with a history of a symptomatic MM. Three months after chemotherapy initiation, she presented with subcutaneous nodules. Ultrasound-guided needle biopsy confirmed the diagnosis of cutaneous plasmacytomas. She underwent local radiation therapy leading to complete regression of subcutaneous nodules. One month later, she developed dyspnoea. Thoracic CT scan showed pleural thickening associated with pleural effusion. Pleural biopsy confirmed the diagnosis of pleural plasmacytoma. Chemotherapy including vincristine, doxorubicin and dexamethasone was administered. Cutaneous involvement and pleural effusion accompanying MM are uncommon. They are associated with poor prognosis.

Fine-Needle Aspiration Cytology of Metastatic Plasmacytoid Urothelial Carcinoma: Report of Four Cases Including a Case of Mixed Plasmacytoid and Micropapillary Morphology.

OBJECTIVES: The aim of this study was to report a small series of fine-needle aspiration (FNA) cytology of the plasmacytoid variant of urothelial carcinoma (PVUC). STUDY DESIGN: A computerized search of our laboratory information system was performed for the 5-year period between January 2008 and January 2013 to identify all FNA cases in which the corresponding surgical pathology cases were diagnosed as PVUC. RESULTS: The 4 cases identified were from 2 men (aged 56 and 64 years) and 2 women (aged 72 and 46 years). The FNA smears demonstrated low-to-moderate cellularity and consisted predominantly of single and dyshesive, medium-sized tumor cells with eccentrically located nuclei and a moderate-to-abundant dense cytoplasm. The nuclei were oval with slightly irregular nuclear membranes and contained coarse granular chromatin with inconspicuous or small nucleoli. There was moderate nuclear variation in size. The nuclear-to-cytoplasmic ratio ranged from <1 to 3. Binucleation, cytoplasmic vacuoles, and perinuclear hof were occasionally seen. CONCLUSIONS: FNA cytology of PVUC shares features with plasma cell neoplasms, lobular carcinoma of the breast, and signet ring cell carcinoma of the stomach. Being aware of the patient's clinical history and the potential diagnostic pitfall of this rare variant of urothelial carcinoma is important for an accurate diagnosis on FNA biopsy.

The Detection of Pancreatic and Retroperitoneal Plasmacytoma Helped to Diagnose Multiple Myeloma: A Case Report.

Multiple myeloma is characterized by the neoplastic proliferation of a single clone of plasma cells producing a monoclonal protein. However, the involvement of pancreas is a rare event. We herein report a rare case of pancreatic plasmacytoma, which was detected before the diagnosis of multiple myeloma.An 83-year-old male was referred to our hospital for further evaluation of obstructive jaundice and a pancreatic mass. A contrast-enhanced computed tomography (CT) scan revealed solid masses with homogenous enhancement in the pancreatic head and retroperitoneum. The histological findings of the retroperitoneal mass obtained by CT-guided biopsy showed multiple sheets of atypical plasma cells, which were positively immunostained for CD79a, CD138, and the κ light chain. Serum immunoelectrophoresis detected M-component of immunoglobulin A-κ, and the histological findings of the bone marrow revealed an abnormally increased number of atypical plasma cells with irregular nuclei and cytoplasmic vacuolation. The patient was therefore diagnosed to have multiple myeloma involving the pancreas and retroperitoneum. Although chemotherapy was performed, the patient died 6 months after the diagnosis.The pancreatic plasmacytoma was detected before the multiple myeloma in the present case. It is difficult to diagnose a pancreatic plasmacytoma without a history of multiple myeloma and related disease.

The t(11;14)(q13;q32) translocation as a poor prognostic parameter for autologous stem cell transplantation in myeloma patients with extramedullary plasmacytoma.

INTRODUCTION: Fluorescence in-situ hybridization (FISH)-detected abnormalities, including del(17p), del(13q), and t(4;14), have been associated with inferior prognosis. However, there are few data about the prognostic significance of cytogenetic abnormalities for autologous stem cell transplantation (ASCT) in multiple myeloma (MM) patients with extramedullary plasmacytoma (EMP). PATIENTS AND METHODS: Between April 2004 and December 2012, 290 MM patients underwent ASCT at 3 centers. FISH data for bone marrow samples obtained at diagnosis were available for 58 patients who had EMP at diagnosis or during treatment. RESULTS: The t(11;14), t(4;14), del(13q), and 1q gain abnormalities were seen in 14.9%, 6.3%, 25.6%, and 42.9%, respectively. No t(14;16) or del(17p) cytogenetic abnormality was detected in the examined patients. Patients with t(11;14) had a lower response rate compared to patients with other cytogenetic abnormalities. EMP-specific relapse was higher in patients with t(11;14) than in patients with other cytogenetic abnormalities (42.9% vs. 10%-33.3%). Each of the 4 cytogenetic abnormalities predicted shorter median progression-free survival (6-12 months vs. 27-37 months) and shorter overall survival (16-22 months vs. 68 months or not reached) compared to no cytogenetic abnormality. The t(11;14) translocation was an important prognostic factor for both progression-free survival (hazard ratio, 25.154; P < .001) and overall survival (hazard ratio, 7.484; P = .024) in the multivariate analysis. CONCLUSION: In the current study, t(11;14), t(4;14), del(13q), and 1q gain were associated with worse survival in MM patients with EMP. The role of t(11;14) as a prognostic parameter for ASCT in MM patients with EMP should be confirmed with a large, well-designed study.

Mieloma múltiple con plasmocitoma extramedular cutáneo abscedado / Multiple myeloma with abscessed cutaneous extramedullary plasmacytoma

Se presentó un paciente con los diagnósticos de bronconeumonía bacteriana extrahospitalaria y anemia para estudio. Se describen la evolución clínica, los estudios imagenológicos, de laboratorio e histológicos que permitieron diagnosticar la presencia de un mieloma múltiple con plasmocitoma extramedular cutáneo abscedado.

A patient with a diagnosis of community-acquired bacterial bronchopneumonia and anemia was presented for study. We describe his clinical course, the imaging, laboratory and histological studies are described. They allowed diagnose the presence of multiple myeloma with abscessed cutaneous extramedullary plasmacytoma.

Resident Rounds: Part III--Case Report: Metastatic Multiple Myeloma.

We report a case of cutaneous plasmacytomas developing in a patient with a 7-month history of progressive multiple myeloma refractory to bortezomib and combination chemotherapy. When involving the skin, plasmacytomas typically arise in the setting of multiple myeloma as contiguous extensions from underlying bony disease. More rarely, cutaneous plasmacytomas develop from hematologic metastases in patients with a high systemic plasma cell tumor burden. In our patient, the presence of cutaneous plasmacytomas involving two distinct sites, and malignant plasma cells within the dermis without infiltration into the subcutaneous fat, suggest a diagnosis of metastatic multiple myeloma to the skin. Metastatic multiple myeloma to the skin portends a poor prognosis, and treatment should be aimed at the underlying systemic disease.

Case for diagnosis. Cutaneous involvement associated to multiple myeloma.

Cutaneous involvement associated to multiple myeloma varies from 5 to 10% of cases and is infrequently recognized. Cutaneous metastatic plasmacitomas are rare. We present the case of a 72-year-old man with multiple myeloma in complete remission since 2 years ago with cutaneous tumors on the trunk and face. A cutaneous biopsy was consistent with plasmacytoma. The patient was treated with melphalan, prednisolone and radiotherapy. Despite optimal therapeutic response of the lesions, the disease progressed, with the appearance of new extra-cutaneous plasmocytomas. The cutaneous metastatic plasmocytomas were the first sign of progression of the disease.

Subglottic extramedullary plasmacytoma with light chain multiple myeloma masquerading as adult-onset asthma.

Extramedullary plasmacytoma (EMP) arises outside the bone marrow and can be associated with multiple myeloma (MM). A 55-year-old gentleman, who presented with dyspnea and expiratory wheeze, was diagnosed and treated for asthma. A subsequent relapse 6 months later prompted an Otolaryngology consult. Preliminary findings showed a benign-looking nodular lesion at the subglottis. Work-up at our institution revealed an Fludeoxyglucose (FDG) avid left subglottic lesion with multiple bone metastases on a Positron Emission Tomography / Computed Tomography (PET/CT). The patient underwent a panendoscopy and laser excision of the subglottic lesion with subglottic jet ventilation. Histology showed an EMP. Further work-up revealed the presence of kappa light chain MM with adverse cytogenetics. Patient was treated systemically with lenalidomide, bortezomib, and dexamethasone for four cycles with rapid improvement in his symptoms. We review the literature about EMP of the subglottis with MM. We present the first case of subglottic laryngeal EMP with MM managed via CO2 laser excision.

Case for diagnosis

Cutaneous involvement associated to multiple myeloma varies from 5 to 10% of cases and is infrequently recognized. Cutaneous metastatic plasmacitomas are rare. We present the case of a 72-year-old man with multiple myeloma in complete remission since 2 years ago with cutaneous tumors on the trunk and face. A cutaneous biopsy was consistent with plasmacytoma. The patient was treated with melphalan, prednisolone and radiotherapy. Despite optimal therapeutic response of the lesions, the disease progressed, with the appearance of new extra-cutaneous plasmocytomas. The cutaneous metastatic plasmocytomas were the first sign of progression of the disease.

Extramedullary plasmocytoma relapsing at differents sites: an unusual presentation.

Extramedullary plasmacytoma (EMP) is an uncommon plasma cell neoplasm results from plasma cell proliferation and consists of monoclonal plasmacytic infiltration, without bone marrow involvement and any other systemic characteristics of multiple myeloma. EMP accounts for 3% of all plasma cell neoplasms and approximately 80% to 90% of EMP involve submucosa of the upper aerodigestive, while scrotal, dermis and retroperitoneal infiltration are very rare. There are no consensus guidelines for treatment, but EMP is highly radiosensitive, surgery may be considered for some sites, but 11 at 30% can progress in multiple myeloma. We report here an exceptional case of recurrent EMP in much localization. It's about a man 72 years old with initially testicular plasmocytoma who generalized the plasmacytic infiltration after 16 months in skin and progressively in mediastinal and retroperitoneal plasmacytoma, without any medullar and bone involvement.

Cutaneous extramedullary plasmacytoma: clinical, prognostic, and interphase cytogenetic analysis.

Extramedullary plasmacytoma (EMP) of the skin is a rare indolent neoplasm that shares morphological and immunophenotypic features with plasma cell myeloma (PCM), but the molecular features that distinguish these two entities have not been defined. We reviewed the clinical characteristics, course, and molecular abnormalities in 7 cases of cutaneous EMP (cEMP); 2 patients had primary cEMP and 5 had secondary cEMP. Two patients died of progressive extramedullary plasmacytoma, 1 without PCM; 1 patient who had only a hyperdiploid clone, died within 17 months of the diagnosis of cEMP; and 3 died of PCM. One patient, who had cEMP with a hyperdiploid clone and a 13q deletion, was alive 28 months after diagnosis. Our findings raise questions about the relative prognostic value of molecular aberrations observed in cEMP and PCM. The role of fluorescence in situ hybridization testing in predicting disease progression of cEMP remains to be defined.