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.

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.

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.

Recurrent multiple myeloma presenting as a breast plasmacytoma.

We describe a patient with multiple myeloma, who initially responded to chemotherapy and went into remission. She presented 10 months later with a right breast lump which was confirmed by core biopsy to be a plasmacytoma. Further treatment with radiotherapy, thalidomide and later second line chemotherapy appeared unsuccessful and she showed rapid disease progression with rising paraproteins and new extramedullary plasmacytoma lesions in the forehead, supraclavicular region, nasopharynx, liver, spleen, pancreas and paraaortic lymph nodes.

Cutaneous metastatic plasmacytomas with tropism for a previously injured limb.

Cutaneous plasmacytoma is an uncommon observation in clinical practice. It is usually a consequence of direct extension from an underlying bony lesion, in the setting of multiple myeloma. In our case, a 77-year-old woman, with stage IIIA IgG lambda multiple myeloma for two years, presented with firm nodular violaceous cutaneous lesions on the left arm without underlying bone osteolytic lesions or subcutaneous tumors; the biopsy was consistent with plasmacytoma. The patient had suffered two spontaneous left humeral fractures treated with prosthesis replacement just before the initial diagnosis of multiple myeloma. She had also been submitted to radiotherapy for a subcutaneous plasmacytoma, detected some months before, at the same site of the cutaneous lesions. Despite optimal response of the cutaneous lesions to treatment, the disease progressed and the patient died from infectious complications eight months after the appearance of the tumors.

Metastatic cutaneous plasmacytoma presenting as a perianal giant mass.

There are 4 types of plasma-cell neoplasia: classic multiple myeloma (MM), extramedullary plasmacytoma without MM, solitary plasmacytoma of bone, and plasma-cell leukemia. Cutaneous involvement may be seen in all 4 types of plasma-cell neoplasia. Specific cutaneous involvement in patients with MM is very uncommon. It usually occurs in late stages of MM as a reflection of increased tumor cell burden. Extramedullary plasmacytoma (EMP) of the skin is a well-recognized, extremely rare, occurrence in MM. Extramedullary plasmacytomas of the skin can be divided into primary cutaneous plasmacytoma (PCP) and metastatic cutaneous plasmacytoma (MCP). Primary cutaneous plasmacytoma is defined as monoclonal proliferation of plasma-cells that arises primarily in the skin without evidence of systemic disease. In contrast to PCP, MCP arises from lymphatic or vascular spread of tumour or, more frequently, by direct extension from underlying bone lesions. Chest, back and abdomen are the most frequently involved areas followed by face, scalp, neck and extremities. Unusual localizations have been described related to MCP, including scrotum, eyelid and tongue. In literature, no cases of MCP presenting as a perianal giant mass have ever been reported. To our knowledge, we describe the first case of MCP located on the perianal area.

[Extramedullary plasmacytoma of the colon]. / Plasmocitoma extramedular de localización colónica.

We report the case of a 68 year-old man in whom a tumour of the colon was identified by colonoscopy, during diagnostic studies for lower gastrointestinal bleeding as an outpatient. Histological examination showed clonal proliferation of plasma cells IgG-K. No other location was affected (including bone marrow). Diagnosis of plasmacytoma of the colon was made. We have carried out a review of the literature in relation to this unusual disorder.

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.

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.

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.

Plasmacytoma in the breast with axillary lymph node involvement: a case report.

Plasmacytic tumors in the breast are extremely rare neoplasms that can mimic mammary adenocarcima. A literature review revealed approximately 43 reports worldwide of plasmacytic tumors in the breast since 1928, the majority with a synchronous diagnosis of multiple myeloma. We discuss management of a 78-year-old man with plasmacytoma in the breast who underwent a left total mastectomy and axillary lymph node dissection. This report characterizes the histologic and immunochemical characteristics of plasmacytoma in the breast and reviews the consensus on treatment. This could be the first recorded account of plasmacytomas in the breast with axillary lymph node involvement and extracapsular extension of tumor.

[Extramedullary plasmacytoma of the nasopharynx. Typical versus atypical presentation]. / Plasmocitoma extramedular de nasofaringe. Presentación tipica versus presentación atípica.

Extramedullary plasmacytoma are rare plasma cell neoplasias. Eighty percent of these tumours grow in the ENT region. We report a typical case of a woman with a single tumour in the nasopharynx. Actually she is in complete remission after surgical and radiotherapic treatment. We also report an atypical case of a woman with a tumour in the nasopharynx associated with a cervical metastatic adenopathy and a plasmacytoma of bone. The treatment of this widespread disease was systemic chemotherapy. An update on this subject is done.

[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.

Plasmacytoma of the breast. An unusual case of recurrent myeloma.

An unusual case of multiple myeloma is presented in which recurrence of the disease after a four-year remission was heralded by an infiltrating plasmacytoma of the breast. The clinical and histologic features of this neoplasm are presented and compared with ten previously reported cases of plasmacytoma of the breast.

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 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.

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.