Unusual coexistence of extramedullary plasmacytoma and nasopharyngeal carcinoma in nasopharynx.

Nasopharyngeal carcinoma (NPC) is an EBV-associated malignant tumor of nasopharynx. As extremely rare condition, the second primary cancer of nasopharynx can occur in NPC patients synchronously or subsequently. Extramedullary plasmacytoma (EMP) is a rare tumor and commonly originates in the head and neck region. However, there is no report to describe a collision tumor of NPC and EMP occurring in the same nasopharyngeal mass. We report here an unusual case of synchronous coexistence of NPC and EMP occurring in the nasopharynx of an old male patient. A 63-year-old male patient presented with a 3-month history of right-sided nasal obstruction and recently intermittent epistaxis without enlargement of cervical lymph nodes. The solitary mass of nasopharynx was found by radiological and nasopharyngeal examination. Histologically, the mass contained two separated portions and displayed typically histological features of NPC and EMP, respectively. In EMP portion, the tumor was composed of monomorphic plasmacytoid-appearing cells with immuno-positive to CD79a, CD138, CD38, MUM-1 and CD56, but lack immunoreactivity to pan-CK (AE1/AE3), CD20, CD21 and EBERs. In NPC portion, the tumor cells formed irregular-shaped islands with diffusely immuno-positive to pan-CK (AE1/AE3), EMA and EBERs, but lack expressions of lymphoplasmacytic markers. A diagnosis of simultaneous occurrence of EMP and NPC in nasopharynx was made. There was no evidence of tumor recurrence or metastasis 18-month follow-up after radiotherapy. To our knowledge, it may be the first case of coexistence of EMP and NPC synchronously. In addition, the histological differential diagnosis and relevant potential mechanism of this unusual collision tumor were also discussed.

Prognostic factors of laryngeal solitary extramedullary plasmacytoma: a case report and review of literature.

A paucity of data exists concerning the presentation, natural course and outcome of extramedullary plasmcytoma (EMP). It is difficult to determine the optimal treatment strategy and prognostic factors for EMP. We present an additional case of laryngeal EMP and systemic review relevant reports in the English and Chinese literature. We found, to our knowledge, 147 cases in larynx in the English-language literature and Chinese-literature. The most common treatment modality was radiotherapy alone. The mean survival duration was ~184 months, and the 5- and 10- year survival rates were 76.1% and 67.4%, respectively. The univariate analysis suggested that progression to multiple myeloma and amyloid deposits may be poor prognostic factors. The multivariate analysis suggested that only progression to multiple myeloma may be a poor prognostic factor. Laryngeal EMP is uncommon. Progression to multiple myeloma may be a poor prognostic factor.

Solitary extramedullary plasmocytoma of the thyroid: a case report and histological approach to plasma cells infiltrate in the thyroid gland.

BACKGROUND: Solitary extramedullary plasmacytoma (SEP) is a rare malignant neoplasm arising from plasma cells. SEP mostly occurs in the upper respiratory tract. Thyroid gland is rarely affected (<78 cases). METHODS/RESULTS: We describe the case of a 78-year-old woman presenting a rapidly enlarging palpable thyroid mass. Neck computed tomography scan showed enlargement of both thyroid lobes. Laboratory tests were normal, including serum protein level with no monoclonal gamma globulin peak. Cytology was suspicious for lymphoma. Biopsy showed an infiltrating neoplasm composed of atypical tumor cells with abundant cytoplasm and eccentric nuclei. These revealed diffuse immunoreactivity for CD138 and predominant staining for immunoglobulin kappa light chains. Clinical workup for multiple myeloma was negative. CONCLUSIONS: SEP should be considered in the differential diagnosis of a rapidly enlarging thyroid nodule and be distinguished from involvement of thyroid in multiple myeloma, mucosa-associated lymphoid tissue lymphoma, plasma cell granuloma and medullary carcinoma. Clinical correlation and immunohistochemistry are crucial in avoiding pitfalls.

Imaging findings of primary gastric plasmacytoma: a case report.

Primary gastric plasmacytoma (GP) is a rare extramedullary plasmacytoma with clinical and imaging features that are common among other gastric tumors, such as gastric adenocarcinomas, gastric stromal tumors, and lymphomas. Here, we present a histologically confirmed case of primary GP examined with biphasic computed tomography (CT), magnetic resonance imaging (MRI), and endosonography. A well-circumscribed extraluminal mass appearing as homogeneous attenuation/intensity with gradual enhancement was identified on biphasic enhancement CT and MRI. This mass was hyperintense on diffusion-weighted imaging and hypointense on the apparent diffusion coefficient map, implying that water diffusion in the mass was restricted. In addition, endosonography indicated a low echogenic mass in the gastric wall. These imaging findings increase the available knowledge about imaging of this disease and provide valuable information for differentiating primary GP from common gastric tumors.

Solitary plasmacytoma of the index finger.

INTRODUCTION: Solitary osseous plasmacytoma rarely involves the distal extremities. We report a case and provide a brief review of the relevant literature. CASE PRESENTATION: We report a 64-year-old man who presented with swelling, mild pain and a deformed right index finger. The workup led to the diagnosis of solitary osseous plasmacytoma and the patient eventually required amputation of his finger. With clinical follow-up, the disease spread to regional lymph nodes and subsequently the patient developed systemic involvement and received chemotherapy. CONCLUSIONS: Solitary osseous plasmacytoma should be considered in the differential diagnosis of distal extremity neoplasms.

Primary extramedullary plasmacytoma of the penis: a case report.

Extramedullary plasmacytoma involving the penis is extremely rare. Here, we describe a case of primary extramedullary plasmacytoma of the penis in a 64-year-old man who presented with a palpable penile mass. Nuclear magnetic resonance imaging revealed the presence of a large, round non-encapsulated mass in the perineum. A contrast-enhanced computed tomography scan of the pelvis showed that the mass was located in the tunica albuginea and corpora cavernosa at the base of the penis. The mass encased the urethra and demonstrated no marked enhancement during the arterial phase. The patient underwent successful surgical resection of the tumor. Histologically, the tumor was composed primarily of neoplastic plasma cells that were positive for CD38, vimentin and Ki 67. Postoperatively, the patient recovered well and exhibited no evidence of development of multiple myeloma, local recurrence or distant metastasis at 2 months post-surgery. To the best of our knowledge, our case represents the first documented case of human primary extramedullary plasmacytoma of the penis.

Primary submucosal nodular plasmacytoma of the stomach: a poorly recognized variant of gastric lymphoma.

Gastric plasmacytoma (GP) is a rare variant of gastric lymphomas. In the exceptional event that a patient presents with GP, the lesion occupies the mucosal layer in the vast majority of cases. Here we report a case of nodular plasmacytoma confined to the submucosa with no evidence of Helicobacter pylori (Hp) infection. The patient was a 59-year old female presenting with no particular symptoms. The tumor was well-demarcated and consisted of a diffuse monomorphic proliferation of plasma cells with numerous lymphoid follicles scattered throughout the tumor. The mucosal surface was intact and not associated with any tumor nodules. The cells were diffusely positive for CD79a, Bob1, EMA and IgA and consistently negative for CD3, CD19, CD20, PAX5, CD56, IgM and IgG. Additionally, in situ hybridization demonstrated clonality in the form of λ light-chain restriction. This submucosal nodular proliferation pattern of plasmacytoma is poorly recognized and considered to be a novel variant of lymphoma. VIRTUAL SLIDES: The virtual slide(s) for this article can be found here: http://www.diagnosticpathology.diagnomx.eu/vs/3489998708673079.

Large neglected ulcerated melanoma mimicking extramedullary plasmacytoma.

Amelanotic melanoma, a renowned impersonator, has taken on a new persona. A 63-year-old woman was seen in the emergency room with a chief complaint of back pain after a fall and was discovered to have a 15-cm fungating mottled gray mass independent of bone on the right elbow. Initial workup discovered lytic calvarial lesions, anemia (Hb 7; Hct 20%), and circulating plasma cells consistent with plasma cell myeloma. Biopsy of the elbow mass displayed sheets of plasmacytoid cells, some reactive for CD138. Flow cytometry revealed a substantial portion of the plasma cells in the tumor that were kappa restricted consistent with cutaneous plasmacytoma. The elbow mass was initially signed out as extramedullary involvement by her myeloma. Reevaluation of the mass after the patient experienced an explosive growth of multinodular jet black malignant melanoma on ipsilateral breast revealed MART-1 and S-100 reactivity of the majority of the cells. In retrospect, the elbow mass was a neglected primary amelanotic malignant melanoma with neoplastic plasma cells participating in its chronic inflammatory infiltrate.

[Extramedullary plasmacytoma of the larynx. A case report]. / Plasmocitoma extramedular de laringe. A propósito de un caso.

Extramedullary plasmacytoma of the larynx and localized laryngeal amyloidosis are two entities that are extremely rare in children. We report the case of an 11-year-old child presenting with progressive dysphonia, with a diagnosis of extramedullary plasmacytoma and localized laryngeal amyloidosis. The treatment he received and subsequent follow-up were compared with the few cases found in the literature.

Plasmacytoma of the kidney.

Extramedullary plasmacytoma is a rare malignant neoplasm typically arising outside the bone marrow of patients who show no clinical evidence of multiple myeloma. Kidney is a rare site for plasmacytoma. We present here a case of primary renal plasmacytoma confirmed on histopathology of the specimen and immuno-histochemistry studies. Patient was treated with radical nephrectomy followed by radiotherapy. The case is presented due to its rarity.

Extramedullary plasmacytoma of the tonsil diagnosed by fine-needle aspiration cytology.

A case of tonsillar extramedullary plasmacytoma in a 53-year-old man with a complaint of lump sensation in the throat is presented. Examination of the oral cavity showed enlargement of the left tonsil. Magnetic resonance imaging demonstrated a solid mass, measuring 3.2 x 2.0 x 3.8 cm, in the left tonsil. Cytologic smear obtained by fine-needle aspiration biopsy appeared highly cellular and was composed of clusters of plasma cells with varying maturity. Atypical plasma cells had prominent eccentric nuclei with nucleoli and finely granular cytoplasm. Binucleated cells and mitotic figures were also identified. The cytoplasm of mature-looking small plasma cells was also finely granular without a perinuclear halo. A cytologic diagnosis of plasmacytoma was made. Excisional biopsy showed sheets of plasmacytoid cells with abundant eosinophilic granular cytoplasm. Occasional binucleated and pleomorphic cells with giant nuclei and prominent nucleoli were observed. These plasmacytoid cells were diffusely immunoreactive for lambda light chain and IgG, partially positive for epithelial membrane antigen. Metastatic examination finding was negative for multiple myeloma, and the patient was diagnosed as having extramedullary plasmacytoma. Although the diagnosis of plasmacytoma on cytologic smear may be difficult, in the current case, fine-needle aspiration cytology provided a rapid and accurate diagnosis.

Sequential bilateral solitary extramedullary plasmacytoma of the ciliary body.

PURPOSE: To report bilateral plasmacytoma of the ciliary body in a healthy patient. METHODS: Clinicopathologic report. RESULTS: A 55-year-old woman with iritis developed an iridociliary mass in the left eye. Systemic evaluation was normal. Fine-needle aspiration (FNAB) of the mass revealed atypical, binucleate plasmacytoid cells with positive leukocyte common antigen staining, suggestive of plasmacytoma. The tumor was treated with 4000 cGy by using custom-designed plaque radiotherapy. The tumor completely resolved. Two years later, a similar iridociliary mass was noted in the right eye, and FNAB confirmed plasmacytoma. Plaque radiotherapy of 4000 cGy was delivered. At the 3-year follow-up, there has been no local recurrence or evidence of systemic multiple myeloma or monoclonal gammopathy. CONCLUSIONS: Extramedullary plasmacytoma can involve the uvea and rarely manifest multiplicity. Long-term monitoring for systemic plasma cell dyscrasia is warranted.

Primary extramedullary plasmacytoma of the esophagus.

An uncommon manifestation of plasma cell neoplasia occurs outside the bone marrow and is designated "extramedullary plasmacytoma." These are usually encountered in mucosal sites of the head and neck region. The gastrointestinal tract may be secondarily involved in multiple myeloma or be the site of primary extramedullary plasmacytomas (PEMPs). The esophagus is the least common site of gastrointestinal PEMP. A 58-year-old man presented with dysphagia for solids over a period of 2 months. Otherwise, he was well and systemic examination did not reveal anything of note. After a nondiagnostic biopsy, the patient was subjected to esophago-gastrectomy. Gross examination of the esophagus revealed a large polypoid tumor. Histologically, it was composed of mature plasma cells, plasmablasts (some of which appeared anaplastic), and a minor admixture of lymphoid cells. Focally, the infiltrate permeated the squamous epithelium simulating lymphoepithelial lesions. The neoplastic cells were positive for epithelial membrane antigen, CD79a, IgG, and kappa, while the lymphoid cells were predominantly B cells. The patient did not have a monoclonal gammopathy. Skeletal x-rays and bone trephine examination were both normal. PEMP is biologically and prognostically different to other plasma cell neoplasms. Although rare, esophageal PEMP should be considered in the differential diagnosis of so-called undifferentiated malignant tumors of the esophagus.

Plasmablastic lymphoma in HIV+ patients: an expanding spectrum.

AIM: To describe an unusual human immunodeficiency virus (HIV)-associated lymphoma in uncommon sites. Plasmablastic lymphoma is a distinctive HIV-associated tumour that was first described in the jaws and oral cavity. Only two cases (stomach and lung) have been documented in extra-oral sites. MATERIALS AND METHODS: Four cases were encountered in HIV+ patients: three in the anorectal region and one which was nasal and paranasal. The cases were routinely processed and immunohistochemistry was performed on formalin-fixed paraffin-embedded tissue. RESULTS: The cases showed the typical morphological appearances of a high-grade, blastic non-Hodgkin's lymphoma (brisk mitotic activity and tingible body macrophages). In addition, some cells had a plasmacytoid appearance and paranuclear clearing. Immunophenotypically, the tumour cells were negative for LCA, CD20 and CD45RA. However, a small proportion of cells (5%) were immunoreactive for CD79a and the majority were positive with VS38c, indicative of plasma cell differentiation. Kappa light chain and IgG heavy chain restriction was also detected. CONCLUSIONS: Plasmablastic lymphoma may occur in extra-oral sites and has a characteristic immunophenotype including focal expression of CD31 by the neoplastic cells. Awareness of the absence of expression of conventional B-cell markers and its presence in unusual sites should facilitate the diagnosis of plasmablastic lymphoma in HIV+ patients.

Anaplastic plasmacytoma with malignant pleural effusion lacking evidence of monoclonal gammopathy.

A case of plasmacytoma of the pleural cavity is reported with massive malignant pleural effusion, which, most unusually, lacked monoclonal gammopathy, thereby making it difficult to distinguish from lymphoma. The pleural tumor and pleural effusion contained large mononuclear lymphoma-like cells with distinct nucleoli. Immunohistochemistry revealed neither lymphoma markers nor clonal cytoplasmic nor cell surface immunoglobulins. Tumor cells were stained with vimentin and the plasma cell markers, VS38c, CD138 (syndecan-1), and MUM1 antibodies. Bone marrow contained small amounts of tumor consisting of similar cells. Electron microscopy showed well developed rough endoplasmic reticulum and peripherally positioned nuclei with euchromatin. Flow cytometry of bone marrow revealed a minimal involvement of CD38-positive cells. Chromosomal analysis of marrow cells revealed a complex abnormal karyotype. A polymerase chain reaction demonstrated clonal re-arrangement of the immunoglobulin heavy-chain gene. The overall results indicate a clonal expansion of tumor cells with primitive plasma cell differentiation with the highly unusual feature of absent monotypic immunoglobulin. The study illustrates the need for a comprehensive array of techniques to distinguish such rare non-synthesizing and non-secretory plasmacytomas from lymphoma.

Extramedullary plasmacytoma: clinical and histopathologic study.

PURPOSE: To review the histories of extramedullary plasmacytoma patients diagnosed in Slovenia between 1969 and 1999, to determine the relationship between radiotherapy (XRT) dose and local tumor control, and to clarify the role of elective nodal XRT and the prognostic value of Bartl's histologic grading criteria (originally devised for multiple myeloma [MM]). METHODS AND MATERIALS: The database of the Cancer Registry of Slovenia was used for the identification of patients. The inclusion criteria were as follows: bone marrow biopsy showing less than 10% plasma cells, normal skeletal survey, and immunohistochemically determined tumor monoclonality. Simulation/portal films were reviewed to assess the extent of elective nodal XRT. RESULTS: Twenty-six patients with 31 tumors fulfilled the inclusion criteria. In 4 patients, nine metachronously appearing solitary tumors were diagnosed. The head-and-neck region and other body sites were the sites of origin of primary tumors in 84% and 16% of patients, respectively, whereas in the two regions, regional disease was seen in 15% and 60% of patients, respectively. Therapy was as follows: XRT, 12 patients; surgery and postoperative XRT, 15 patients; and surgery, 4 patients. Ultimate local and regional control rates were 90% and 97%, respectively, and MM developed in 2 (8%) patients. The 10-year disease-specific and overall survival rates were 87% and 61%, respectively. The analysis of the dose-effect relationship showed that more conservative treatment is justified: for macroscopic disease, 40-50 Gy (2 Gy/day), adjusted to the bulk of disease; for microscopic disease, 36-40 Gy; after R0 surgery, no XRT is required, but close observation is needed. No attempts should be made to treat uninvolved nodal regions. Using Bartl's histologic grading criteria, trends were detected in patients with higher tumor grades: regional lymph node involvement (p = 0.04) and shorter disease-specific survival (p = 0.08). CONCLUSIONS: Extramedullary plasmacytoma is a highly curable disease when XRT is used with or without previous surgery. The rate of conversion to MM is low. Moderate-dose XRT using limited fields is recommended. The prognostic value of Bartl's grading system needs further evaluation.

[Syndecan-1 (CD138): an immunohistochemical marker of plasma cell tumors]. / Syndecan-1 (CD138): imunohistochemický marker nádoru z plazmatických bunek.

The immunohistochemical detection of syndecan-1 (belonging to the cluster CD138) is a sensitive and reliable method for identifying normal and neoplastic plasma cells. It may be used in paraffin-embedded bone marrow specimens, as well as in extramedullary tumours of unknown origin. The three anaplastic tumours reported by us in the lymph node, the gingiva, and pleura were negative for other markers, but the syndecan-1 positivity elucidated their plasmocytic histogenesis.

Hyposialated 185 kDa CD45RA+ molecules attain a high concentration in B lymphoma cells and in activated human B cells.

Alternate splicing of exons of the CD45 molecule generates multiple isoforms differing in their molecular weights (MWs). In B-lymphocytes the CD45RA isoform was previously shown to be expressed on glycoproteins with MWs of 220 and 205 kDa, while the CD45RO isoform was expressed on glycoproteins with MW of 180 kDa. The present study demonstrated that B cell lymphomas and activated B-cells contain CD45 molecules with a MW of 185 kDa that express the CD45RA and CD45RC specificities but neither the CD45RB nor the CD45RO specificities. 185 kDa CD45RA+ molecules were detected in B cell lymphoma B lines, in Epstein-Barr virus (EBV)-transformed lymphoblastoid cell lines, and in tonsillar B cells, but not in normal, unstimulated peripheral blood B cells. These molecules were not detected in neoplastic and normal T cells. CD45RA+ 185 kDa molecules were present in B cells from three non-Hodgkin's patients in leukemic phase were not detected in B lymphocytes of seven of nine CLL patients tested. Trypsin treatment eliminated only 220 kDa CD45RA+ molecules but not 185 kDa CD45RA+ molecules, indicating that the 185 kDa CD45RA+ molecules are not expressed on the cell surface. Pulse-chase experiments, and studies on the effects of tunicamycin, neuraminidase and O-glycosidase, indicated that the 185 kDa molecules are partially glycosylated CD45RABC molecules that constitute precursors of the 220 kDa molecules. The high concentration of 185 kDa CD45RA+ molecules in B lymphoma cells and in activated B cells seems to reflect a high turnover of CD45RA+ molecules characteristic for these cells.