Plasmacytoma is a localized collection of monoclonal plasma cells. The disease is divided into primary plasmacytoma of the bone and extramedullary plasmacytoma. The importance of the diagnosis rests with the potential for these disorders to progress to multiple myeloma. Solitary plasmacytoma of the bone are usually painful and present with a lytic lesion on radiographs. By definition, the bone lesion can be diagnosed if there is:
Single bone lesion with histology consistent with a plasma cell tumor
Absence of a plasma cell infiltrate in random bone marrow biopsies
No evidence of other bone lesions by radiographic examination
Absence of renal failure
The pathologist plays an important role not only in performing the bone marrow aspiration and biopsy but evaluating it under the microscope for the presence of plasma cells and finally determining whether these plasma cells are monoclonal.
Epidemiology Disease Associations Pathogenesis Laboratory/Radiologic/Other Diagnostic Testing Gross Appearance and Clinical Variants Histopathological Features and Variants Special Stains/
Differential Diagnosis Prognosis Treatment Commonly Used Terms Internet Links
EPIDEMIOLOGY CHARACTERIZATION SYNONYMS None INCIDENCE 5% of patients with multiple myeloma present with a solitary plasmacytoma AGE-RANGE AND MEDIAN Median 50-55 years SEX (MALE:FEMALE) Male 70% GEOGRAPHIC DISTRIBUTION Worldwide
DISEASE ASSOCIATIONS CHARACTERIZATION
- Primary Cutaneous Plasmacytoma (Posttransplant Lymphoproliferative Disorder, Plasmacytoma-like) in a Heart Transplant Patient.
From the Department of Pathology, Scott and White Memorial Hospital and Clinic Scott, Sherwood and Brindley Foundation; and The Texas A&M University System Health Science Center College of Medicine, Temple, Texas.
- Am J Dermatopathol. 2006 Oct;28(5):442-5 Abstract quote
Extramedullary plasmacytomas in posttransplant patients are rarely encountered.
We present the fifth case of a primary cutaneous plasmacytoma (posttransplant lymphoproliferative disorder, plasmacytoma-like) that developed in a heart transplant patient. The tumor presented as a solitary nodule of the skin 10 years after transplantation. It subsequently involved 2 other cutaneous sites and remained confined to the skin for 5 years.
The neoplastic cells were Epstein-Barr virus small RNAs (EBER 1,2) positive and EBV-latent membrane protein 1 (LMP 1) negative, corresponding to type I EBV latency. The direct role of EBV in the development of the tumor remains uncertain.
CHARACTERIZATION Bone Solitary lytic lesion on radiograph Serum and urine protein electrophoresis <50% have a detectable monoclonal protein in serum or urine
Monoclonal protein may disappear with removal of the tumor
CHARACTERIZATION GENERAL BREAST
Primary Plasmacytoma of the Breast
Annarosaria De Chiara, MD, Simona Losito, MD, Luigi Terracciano, MD, Raimondo Di Giacomo, MD, Giancarla Iaccarino, PhD, and Maria R. Rubolotta, MD
From the Department of Pathology (Drs De Chiara and Losito), Division of Surgical Oncology D (Dr Di Giacomo), Division of Hematology (Dr Iaccarino), and Department of Radiology (Dr Rubolotta), Istituto dei Tumori “G. Pascale” di Napoli, Italy; and Institut für Pathologie, Kantonsspital Basel, Switzerland (Dr Terracciano).
Arch Pathol Lab Med 2001;125:1078–1080. Abstract quote
We describe a solitary extramedullary plasmacytoma of the breast in a 37-year-old woman. No other involvement was detected in the bone marrow or in any other site during a 15-month follow-up period. Extramedullary plasmacytomas of the breast are extremely rare, especially those that are not associated with multiple myeloma.
We review the histologic features of the previously reported cases with an emphasis on differential diagnosis and the difficulties encountered in arriving at the correct diagnosis in frozen sections.
Primary extramedullary plasmacytoma of the esophagus.
Chetty R, Bramdev A, Reddy AD.
Ann Diagn Pathol. 2003 Jun;7(3):174-9. Abstract quote
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.
HEAD AND NECK Most commonly occurs in the mucous membranes of the upper respiratory system
Rarely evolves to multiple myeloma
Plasma cell dyscrasias and the head and neck.
Batsakis JG, Medeiros JL, Luna MA, El-Naggar AK.
Department of Pathology, University of Texas M. D. Anderson Cancer Center, Houston, TX.
Ann Diagn Pathol 2002 Apr;6(2):129-40 Abstract quote
Structures in the head and neck (bones, soft tissues, lymph nodes, mucosa) are variably affected by plasma cell dyscrasias. Involvement can be manifested by localized lesions (extramedullary plasmacytoma or solitary plasmacytoma of bone) or by more diffuse disease (multiple myeloma).
We present a contemporary review of these disorders with emphasis on patient outcomes.
LYMPH NODE Primary lymph node plasmacytoma (Plasmacytic lymphoma)
Am J Clin Pathol 2001;115:119-126
Similar histologic features to extramedullary plasmacytomas
Did not progress to multiple myeloma but survival was similar to other extramedullary tumors
Primary cutaneous plasmacytoma--report of two cases and review of the literature.
Wong KF, Chan JK, Li LP, Yau TK, Lee AW.
Department of Pathology, Queen Elizabeth Hospital, Hong Kong.
Am J Dermatopathol 1994 Aug;16(4):392-7 Abstract quote
Primary cutaneous plasmacytomas are very rare. In this report, we describe two such cases and review the literature on this entity. Both patients presented with a slowly growing, painless, and solitary mass on the chest wall.
Histologically, one case was composed of mature-looking plasma cells, while the other was composed of immature and anaplastic plasma cells, infiltrating the dermis. The epidermis was spared. Kappa light-chain restriction was demonstrated by immunohistochemical techniques in both cases. There was no evidence of marrow disease even on repeated marrow biopsies, although extracutaneous lesions were detected in one patient. One remained in complete remission 6 years following local irradiation. The other patient was treated with local irradiation and systemic chemotherapy, with a complete response, but this was followed by multiple cutaneous recurrences and further remissions by treatment with cytotoxic agents. The present cases and those reported in the literature illustrate well the variable clinical course of primary cutaneous plasmacytoma. Although cure can apparently be achieved in some patients by local radiation therapy, more than half of the cases relapse or progress to myelomatosis.
The disease-related mortality is at least 40%. Thus cutaneous plasmacytoma appears to be more aggressive than noncutaneous extramedullary plasmacytomas and should be separately categorized from them in future studies.
Primary cutaneous plasmacytoma: report of a case with review of the literature.
Muscardin LM, Pulsoni A, Cerroni L.
Dermatologic Institute SS. Maria & Gallicano, Rome, Italy.
J Am Acad Dermatol 2000 Nov;43(5 Pt 2):962-5 Abstract quote
Primary cutaneous plasmacytoma (PCP) is a rare cutaneous B-cell lymphoma.
We report a new case of PCP and review data published in the literature. A 55-year-old man presented with 2 erythematous plaques on the upper trunk, showing histologic and immunohistochemical features of PCP. Staging investigations excluded extracutaneous manifestations of the disease. The patient was treated with melphalan and prednisone associated with local radiotherapy. Twenty-four months after the first presentation he is alive and well. Only 29 cases of PCP have been reported in the last 50 years. The main prognostic factor seems to be the clinical presentation (solitary vs multiple lesions).
Solitary lesions of PCP may be treated conservatively by surgical excision or local radiotherapy.
Primary cutaneous plasmacytoma: a clinicopathological study of two cases with a long-term follow-up and review of the literature.
Kazakov DV, Belousova IE, Muller B, Palmedo G, Samtsov AV, Burg G, Kempf W.
Department of Dermatology, University Hospital, Zurich, Switzerland, Department of Dermatology, Medical Military Academy, Saint-Petersburg, Russia and Dermatopathology Laboratory, Friedrichshafen, Germany.
J Cutan Pathol 2002 Apr;29(4):244-8 Abstract quote
BACKGROUND: Primary cutaneous plasmacytoma (PCP) is a rare type of cutaneous B-cell lymphoma arising primarily in the skin and derived from clonally expanded plasma cells with a various degrees of maturation and atypia. The disease is rare with only 30 cases reported so far.
METHODS: Two cases of PCP with long-term follow-up of 17 and 15 years are presented.
RESULTS AND CONCLUSIONS: Both patients were men with nodular lesions on the face. Histologically, the lesions were composed predominantly of variably maturated plasma cells with monotypic expression of immunoglobulin (Ig) lambda chains. Polymerase chain reaction for IgH genes did not reveal clonal rearrangement. Our cases are discussed in the context of previously reported cases of PCP with a long-term follow-up. We also include a review of all cases of PCP with known tumor progression earlier in the course of the disease (local relapse or visceral spread) to determine the clinical course of this primary cutaneous lymphoma.
HISTOLOGICAL TYPES CHARACTERIZATION General Proliferation of plasma cells which may be mature or immature
Immunoperoxidase studies may establish light chain restriction, establishing monoclonality
Negative for CD20, CD79a, CD3
Occasionally may show positivity for CD43, CK, EMA, and CD31
J Pathol. 2005 Jan;205(1):92-101. Abstract quote
Primary extramedullary plasmacytomas are infrequent, typically solitary, plasma cell neoplasms that generally pursue an indolent clinical course but may, rarely, convert to multiple myeloma. Phenotypic differences between these two entities are not well defined. Twenty-eight cases of primary extramedullary plasmacytoma and 26 cases of both medullary (n = 17) and extramedullary (n = 9) multiple myeloma were analysed for the expression of proteins known to play a role in the biology of multiple myeloma.
Immunohistochemistry was performed on paraffin wax sections using antibodies against cyclin D1, Bcl-2, Bcl-xL, p27, p21, p53, MIB1, CD20, and CD56. Twenty-three extramedullary plasmacytomas were localized in the upper aerodigestive tract, four in the lymph nodes, and one in the testis. There was a strong male predominance (M : F = 6 : 1). None of the patients died from the disease or progressed to multiple myeloma (mean follow-up 50 months). Nine patients developed local relapse and one patient's tumour evolved into a B-cell non-Hodgkin's lymphoma. In contrast to both intra- and extra-medullary multiple myeloma, extramedullary plasmacytoma showed absence of cyclin D1 (p < 0.001) and infrequent expression of CD56 (p < 0.001). Furthermore, extramedullary plasmacytomas were characterized by weaker staining for Bcl-2 protein and rare overexpression of p21 and p53. In comparison to extramedullary multiple myeloma, extramedullary plasmacytoma showed a more mature morphology and lower proliferation indices (p = 0.008).
There was no association between the phenotypic parameters investigated and clinical outcome in extramedullary plasmacytoma. In summary, extramedullary plasmacytoma and multiple myeloma show significant immunophenotypic differences, some of which may be of both diagnostic utility and biological relevance.
Detection of clonality with kappa and lambda immunohistochemical analysis in cutaneous plasmacytomas.
Bayer-Garner IB, Prieto VG, Smoller BR.
Department of Pathology, Marshfield Clinic, Marshfield, Wis 54449, USA.
Arch Pathol Lab Med. 2004 Jun;128(6):645-8. Abstract quote
CONTEXT: Cutaneous plasmacytomas rarely occur in the setting of multiple myeloma. However, since poorly differentiated lesions may resemble other neoplasms, such as carcinoma, melanoma, and lymphoma, the diagnosis of cutaneous plasmacytoma may be difficult.
OBJECTIVE: To demonstrate clonality using kappa and lambda immunohistochemical analysis in cutaneous plasmacytomas and to ascertain whether or not interpretation is hindered by background staining.
DESIGN: Pathology reports of all patients with the diagnosis of multiple myeloma were reviewed. Twelve patients had cutaneous lesions diagnosed as plasmacytoma, and these lesions were analyzed for light chain restriction with kappa and lambda immunohistochemical analysis.
RESULTS: In most cases (11 of 12), monoclonality was demonstrated. In the remaining case, monoclonality could not be established because most cells did not stain for either kappa or lambda.
CONCLUSIONS: Light chain restriction can be demonstrated in most multiple myeloma-related cutaneous plasmacytomas, establishing the neoplastic nature of the infiltrate.
Plasmacytoma with aberrant expression of myeloid markers, T-cell markers, and cytokeratin.
Shin JS, Stopyra GA, Warhol MJ, Multhaupt HA.
Department of Pathology, Pennsylvania Hospital, Philadelphia, Pennsylvania, USA.
J Histochem Cytochem 2001 Jun;49(6):791-2 Abstract quote
Plasmacytomas are localized neoplastic proliferations of monoclonal plasma cells. When multifocal, the process is referred to as multiple myeloma. These lesions exhibit a pattern of antigen expression and cytomorphology that usually leads to a ready diagnosis. However, potentially troublesome variations in immunophenotype occur.
We describe a case of a plasmacytoma from a patient who presented with sudden onset of pain and a lytic lesion of the left proximal humerus. Hematoxylin and eosin-stained sections showed a lymphoproliferative lesion composed of large lymphoid cells, some with plasmacytoid and immunoblastic features. The lesion also showed significant mitotic activity. Immunohistochemical staining was positive for CD45 (LCA), CD56 (N-CAM), CD43 (MT1), and cytokeratin CAM5.2. There was also clonal staining for lambda light chains. In addition, flow cytometric analysis showed positivity for myeloid markers such as CD13, CD33, CD38, and CD138. Significant negative markers include CD20 (L26), CD45RO (UCHL-1), and CD79alpha.
The unusual phenotypic features of this plasmacytoma illustrate potential diagnostic pitfalls. It is important to fully study such lesions to correctly classify them, because this has significant impact on prognosis and management.
J Clin Pathol. 1997 Jun;50(6):490-3. Abstract quote
AIMS: To investigate the immunohistochemical expression of CD31 (JC70) in normal and neoplastic plasma cells.
METHODS: Plasma cells in bone marrow biopsies and extramedullary locations were examined. All extramedullary biopsies were formalin fixed and paraffin embedded. The bone marrow biopsies were fixed in formal acetic acid and embedded in paraffin wax. Twenty multiple myelomas (12 bone marrow and eight extramedullary deposits), 10 extramedullary plasmacytomas, and 30 biopsies with reactive plasma cells (10 bone marrow, 20 extramedullary biopsies) were stained with anti-CD31 (JC70) using the streptavidin-biotin detection system with diaminobenzidine as a chromogen. Antigen retrieval in bone marrow biopsies was achieved by pressure cooking. In all other biopsies, antigen retrieval was achieved by microwave pretreatment.
RESULTS: All 20 extramedullary cases with reactive plasma cells showed intense membrane staining. Focal staining was detected in reactive plasma cells in bone marrow biopsies. Five of 10 plasmacytomas showed membrane staining. None of the cases of multiple myeloma, either medullary or extramedullary, showed any immunoreactivity for CD31.
CONCLUSIONS: CD31, a member of the immunoglobulin supergene family of cell adhesion molecules, is strongly expressed in extramedullary reactive plasma cells, focally in bone marrow reactive plasma cells, and occasionally in extramedullary plasmacytomas.
Arch Pathol Lab Med. 2003 Dec;127(12):1596-8. Abstract quote
CONTEXT: CD117 (c-Kit) and lysozyme are frequently expressed by myeloblasts and are sensitive markers for the diagnosis of extramedullary myeloid tumor. The diagnosis of cutaneous plasmacytoma presents a degree of difficulty, particularly with the plasmablastic variant, which can mimic hematologic as well as epithelioid malignancies. Approximately 25% of multiple myelomas express CD117 in the bone marrow by flow cytometry. Lysozyme immunoreactivity has been previously shown in 30% of poorly differentiated myelomas, while it is nonreactive in nonmalignant plasma cells.
OBJECTIVE: To ascertain whether CD117 and lysozyme can aid in the diagnosis of cutaneous plasmacytomas, particularly the plasmablastic type.
DESIGN: Pathology reports of 2357 patients with a diagnosis of multiple myeloma were reviewed to find 13 cutaneous plasmacytomas (8 Bartl grade II, 5 Bartl grade III). Formalin-fixed, paraffin-embedded tissue sections were stained with CD117 and lysozyme on the Dako Autostainer system.Setting.-Patients with the diagnosis of multiple myeloma who developed cutaneous plasmacytoma(s).
RESULTS: The cutaneous plasmacytomas uniformly expressed CD117 in a cytoplasmic or membranous and cytoplasmic distribution with varying degrees of staining intensity unrelated to the Bartl grade of the lesion, while they were uniformly negative for lysozyme.
CONCLUSIONS: CD117 is a sensitive marker for malignant plasma cells in paraffin-embedded tissue, while lysozyme does not help identify poorly differentiated malignant plasma cells. While CD117 alone does not distinguish extramedullary myeloid tumor from poorly differentiated myeloma, the combination of CD117 and lysozyme may allow their differentiation. The possibility of c-kit inhibitors being used in the treatment of other hematopoietic malignancies allows speculation regarding implications for the treatment of multiple myeloma.
CD138 (SYNDECAN-1) CD138 (Syndecan-1), a Plasma Cell Marker Immunohistochemical Profile in Hematopoietic and Nonhematopoietic Neoplasms
Fionnuala P. O'Connell, MD, Jack L. Pinkus, PhD, and Geraldine S. Pinkus, MD
Am J Clin Pathol 2004;121:254-263 Abstract quote
We evaluated the immunohistochemical profile and specificity of CD138 reactivity in 238 specimens from hematopoietic and nonhematopoietic neoplasms. In 91 bone marrow biopsies, CD138 reactivity was observed for nonneoplastic plasma cells, neoplastic plasma cells in multiple myeloma cases (43/43), and the plasmacytic component in lymphoplasmacytic lymphoma cases (4/4).
Stromal reactivity was noted in 7 multiple myeloma cases. Of 9 bone marrow specimens involved by metastatic carcinoma, tumor cells were CD138+ in 5 cases; stromal reactivity was noted in 7 cases.
Studies of 76 nodal and extranodal lymphomas (B-cell, 49; T-cell, 8; Hodgkin lymphoma, 19), 1 Langerhans cell histiocytosis, and 14 nonneoplastic lymph nodes revealed CD138 reactivity only for nonneoplastic plasma cells, the neoplastic cells of 2 large B-cell lymphomas (immunoblastic type, plasmacytoid features), and the clonal plasmacytic component of 3 of 3 extranodal and 1 nodal marginal zone lymphoma.
Evaluation of 56 epithelial and nonepithelial tumors revealed CD138 positivity for neoplastic cells of carcinomas of various types (30/33), frequently with associated stromal reactivity, and for neoplasms of mesenchymal, melanocytic, and other tumor types (12/23).
Within the hematopoietic system, CD138 is an excellent marker of plasmacytic differentiation. Based on its broad staining profile, CD138 reactivity for neoplastic cells is not a definitive marker for plasmacytic derivation, unless a hematolymphoid origin has been established.
Expression of syndecan-1 is a sensitive marker for cutaneous plasmacytoma.
Bayer-Garner IB, Joseph L, Sanderson RD, Smoller BR.
Departments of Pathology, Dermatology and Anatomy, and the Arkansas Cancer Research Center, University of Arkansas for Medical Sciences, Little Rock, AR, USA.
J Cutan Pathol 2003 Jan;30(1):18-22 Abstract quote
BACKGROUND: Cutaneous plasmacytoma is a well-recognized, yet infrequent, occurrence in multiple myeloma (MM). There are limitations in the morphologic assessment, and as such, the diagnosis presents some difficulty, particularly with the plasmablastic type.
METHODS: Pathology reports of 2357 patients with a diagnosis of MM were reviewed. Twenty patients yielded a total of 25 plasmacytomas, 10 of which were analyzed for syndecan-1 immunoreactivity. Bartl grade of bone marrow and cutaneous plasmacytoma was compared and immunoglobulin secretory status of the patients was assessed.
RESULTS: The incidence of cutaneous plasmacytoma was found to be 1 in 118 patients with MM. Immunoglobulin secretion was found to be predominantly IgG. There was a trend for the plasmacytoma Bartl grade to be equal to or greater than that of the corresponding bone marrow Bartl grade, suggesting a more aggressive phenotype in the metastatic lesion.
CONCLUSION: Syndecan-1 was found to be a sensitive marker for plasmacytomas, independent of cytologic differentiation.
DIFFERENTIAL DIAGNOSIS KEY DIFFERENTIATING FEATURES PLASMACYTOSIS, CUTANEOUS
Am J Dermatopathol. 2006 Apr;28(2):93-98. Abstract quote
Cutaneous plasmacytosis is a rare disorder that typically affects middle-aged to older individuals of Asian, particularly Japanese, descent. Clinically, it is characterized by multiple asymptomatic red-brown plaques and nodules on the trunk. Lymphadenopathy and hypergammaglobulinemia may be present.
Histologically, the lesions show a moderately dense superficial and deep perivascular infiltrate composed predominantly of mature plasma cells without atypia or light chain restriction.
We report our experience with five additional cases, including results of immunohistochemical studies for human herpes virus 8.
Primary cutaneous plasmacytosis: report of three cases and review of the literature.
Uhara H, Saida T, Ikegawa S, Yamazaki Y, Mikoshiba H, Nijoh S, Kitano K, Koh CS.
Department of Dermatology, School of Medicine, Shinshu University, Matsumoto, Japan.
Dermatology 1994;189(3):251-5 Abstract quote
BACKGROUND: Cutaneous plasmacytosis is a rare disease characterized by peculiar multiple eruptions and hypergammaglobulinemia. More than 40 cases have been reported, mainly in Japan, although information concerning the disorder was limited to individual case reports.
OBJECTIVE AND METHODS: To clarify the clinicopathological and laboratory features, we reviewed 41 cases.
RESULTS: All patients were Japanese and the male-to-female ratio was 1:0.6. The onset ages ranged from 20 to 62 years, with a mean and median of 37 and 37 years. A superficial lymphadenopathy was detected in 58% (22/38), and polyclonal hypergammaglobulinemia was found in 93% (38/41). No cases were associated with any apparent underlying diseases. The course was chronic without spontaneous remission. Four patients died, 3 of whom succumbed to leukemia, respiratory failure or renal failure, respectively.
CONCLUSION: The results suggest that the condition appears to be a variant of reactive plasmacytic disorders of unknown origin.
Is cutaneous plasmacytosis a distinct clinical entity?
Shimizu S, Tanaka M, Shimizu H, Han-yaku H.
Division of Dermatology, Ogikubo Hospital, Tokyo, Japan.
J Am Acad Dermatol 1997 May;36(5 Pt 2):876-80 Abstract quote
We describe a Japanese patient with cutaneous plasmacytosis whose clinical course we observed for 5 years. We also review 26 patients with this condition, including 24 Japanese and two non-Japanese, reported in detail.
This review revealed that this condition has characteristic clinical and pathologic features and should be considered a distinct clinical entity. The reason for the predominant occurrence of cutaneous plasmacytosis in Japanese patients is unknown.
Primary cutaneous plasmacytosis in a child. Is this a new entity?
Arico M, Bongiorno MR.
Department of Dermatology, University of Palermo, Italy.
J Eur Acad Dermatol Venereol 2002 Mar;16(2):164-7 Abstract quote
Plasma cell proliferations represent a heterogeneous spectrum of disorders. A 7-year-old Caucasian female had suffered an asymptomatic eruption on the trunk for 4 years. Physical examination revealed a plaque with scattered red-brown papules and nodules.
Chemical analysis revealed normal proteinaemia. Histological examination of biopsy specimens showed dense perivascular and periadnexal infiltrate, consisting largely of plasma cells, in the superficial and deep dermis. Immunohistochemical study showed that many cells of the infiltrate were CD20 positive. The plasma cells expressed kappa and lambda light chains. The girl's status (age; absence of hypergammaglobulinaemia, lymphadenopathy and hepatosplenomegaly; presence of an infiltrate of mature polyclonal plasma cells restricted only to the skin) differed from those generally seen in diseases with plasma cell proliferation reported in the literature.
This case seems unlike any other described up to the present time.
Cutaneous and Systemic Plasmacytosis in a Patient of Asian Descent Living in the United States
Hesham M. Amin, M.D., M.Sc.; Peter McLaughlin, M.D.; Cynthia J. Rutherford, M.B, Ch.B.; Lynne V. Abruzzo, M.D., Ph.D.; Dan Jones, M.D., Ph.D.
Am J Dermatopathol 2002; 24(3):241-245 Abstract quote
Cutaneous and systemic plasmacytosis is a rare disorder characterized by widely disseminated macular skin eruptions composed of polyclonal lymphoplasmacytic infiltrates associated with variable extracutaneous involvement. Previous reports have been largely restricted to the Japanese literature.
We present the first documented case of cutaneous and systemic plasmacytosis in a patient residing in the United States. This 49-year-old man, who had immigrated from Korea 19 years earlier, developed innumerable persistent pink-to-brown macular lesions over his trunk and face. Initial and repeat skin biopsy specimens revealed dense perivascular and periadnexal infiltrates of mature plasma cells, and polyclonal plasmacytosis noted on two different biopsy specimens of mildly enlarged lymph nodes. Multiple tiny pulmonary nodules were found to be of the same histologic appearance. No evidence of clonal immunoglobulin gene rearrangements or human herpesvirus type 8 infection was noted in these biopsy specimens. Treatment with antibiotics, systemic chemotherapy, and anti-CD20 antibody therapy failed to eradicate these lesions, which have persisted for 6 years.
This case demonstrates that cutaneous and systemic plasmacytosis can arise in a patient of Asian ancestry, even many years after emigration to the United States.
T-CELL RICH B-CELL LYMPHOMA
Characteristics of Cutaneous Marginal Zone Lymphomas With Marked Plasmacytic Differentiation and a T Cell–Rich Background
Julia Turbiner Geyer, MD1, Judith A. Ferry, MD1, Janina A. Longtine, MD2, Thomas J. Flotte, MD1,*, Nancy L. Harris, MD1 and Lawrence R. Zukerberg, MD1
1From the Departments of Pathology, Massachusetts General Hospital and
2Brigham and Women’s Hospital, Boston, MA
Address reprint requests to Dr Zukerberg: Pathology Department (Warren 2), Massachusetts General Hospital, 55 Fruit St, Boston, MA, 02114.
Am J Clin Pathol 2010:133:59-69
Primary cutaneous marginal zone lymphoma (MZL) is a common B-cell lymphoma of skin and is characterized by an infiltrate of neoplastic marginal zone B cells typically within the marginal zones of reactive lymphoid follicles and the interfollicular region. However, in our experience, many cases have underemphasized features such as marked plasmacytic differentiation and/or a prominent T-cell component, which may obscure the neoplastic B cells and lead to misdiagnosis.
We wanted to draw attention to these features and have studied 15 cases of MZL with marked plasmacytic differentiation, 10 of which had numerous T cells, some with cytologic atypia, and few B cells in the interfollicular region. Plasma cells were monotypic in all cases by in situ hybridization. By polymerase chain reaction, 6 of 8 T cell–rich cases had an IGH gene rearrangement, and none were clonal for T-cell receptor gene.
We discuss the terminology, morphologic features, molecular profile, behavior, and differential diagnosis of cutaneous MZL.
PROGNOSIS AND TREATMENT CHARACTERIZATION Prognostic Factors
In cases of solitary lesions of the bone, there is progression to multiple myeloma in about 2/3 of cases, within 2-10 years
About 10-20% of solitary extramedullary plasmacytomas will progress to multiple myeloma on follow up
5 Year Survival Median survival exceeds 10 years LYMPHOMATOUS TRANSFORMATION
Epstein-Barr virus-induced transformation of cutaneous plasmacytoma into CD30+ diffuse large B-cell lymphoma.
Department of Pathology, Scott & White Memorial Hospital, The Texas A&M University System Health Science Center, College of Medicine, Temple, TX 76508, USA.
Am J Dermatopathol. 2004 Feb;26(1):63-6. Abstract quote
A unique, previously unreported case of transformation of cutaneous plasmacytoma into CD30+ large B-cell lymphoma is described. Both neoplastic components were immunophenotypically distinct.
The plasma cells were CD20-, CD30-, CD43+, CD45+, lambda +; the blasts were CD20+, CD30+, CD43-, and CD45-. The large B-cell lymphoma has gradually become a predominant component of the neoplastic nodules. While plasma cells and blasts were both positive for Epstein-Barr virus-encoded nuclear RNAs (EBER-1), the EBV-latent membrane antigen 1 (EBV-LMP1) was expressed only in the minority of the blasts and not in the plasma cells.
The neoplastic process has remained confined to the skin for more than six years since its development.
Depends upon the location and clinical symptomatology
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