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Metastatic cutaneous plasmacytoma presenting as a perianal giant mass

  • Author(s): Alvarez-Twose, Iván
  • Vano-Galvan, Sergio
  • Calvo-Villas, José Manuel
  • Carreter, Elena
  • Piqué, Enric
  • Palacios, Santiago
  • et al.
Main Content

Metastatic cutaneous plasmacytoma presenting as a perianal giant mass
Iván Alvarez-Twose1, Sergio Vano-Galvan2, José Manuel Calvo-Villas1, Elena Carreter1, Enric Piqué3, Santiago Palacios4
Dermatology Online Journal 14 (9): 17

1. Department of Hematology, Hospital General de Lanzarote. Arrecife de Lanzarote. Las Palmas, Spain
2. Department of Dermatology, Hospital Ramón y Cajal. Madrid. Spain. sergiovano@yahoo.es
3. Department of Dermatology, Hospital General de Lanzarote. Arrecife de Lanzarote. Las Palmas, Spain
4. Department of Pathology, Hospital General de Lanzarote. Arrecife de Lanzarote. Las Palmas, Spain


Abstract

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.



Introduction

There are 4 types of plasma-cell neoplasia: classic multiple myeloma (MM), extramedullary plasmacytoma (EMP) 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 [1]. Cutaneous plasmacytoma (CP) is a well-recognized, yet infrequent, occurrence in MM. This is most commonly seen as direct extension from a bony focus of disease. However, it can also be seen distinct from a bony focus and even as the initial manifestation of the disease or without evidence of coexisting MM [2]. We report a case of metastatic cutaneous plasmacytoma (MCP) that presented as a giant perianal mass, an unusual presentation.


Case report

A 36-year-old man reported weakness and severe bone pain. A parasternal subcutaneous nodule and a soft, round, well-defined, fresh-colored papule on the scalp of 1cm in diameter were seen on physical examination. Routine laboratory studies revealed increased levels of LDH, hypercalcemia and mild renal failure. Serum and urine immunofixation electrophoresis detected a monoclonal lambda-IgA spike and lambda light chains, respectively. High levels of serum IgA (20 mg/dL) were measured. Serology for HBV, HCV, CMV and HIV were negative. A thoracic-CT showed multiple mediastinal adenopathies and mild bilateral pleural effusion. Subcutaneous nodule biopsy revealed a diffuse infiltration of a population of CD38+, CD138+, CD79a+, and lambda+ plasma-cells. Cyclin D1, EBV, and HHV-8 were negative. Bone scintigraphy revealed lesions in both shinbones suggestive of bone afectation by MM. Diagnosis of MM was confirmed through bone marrow biopsy that showed an infiltration of atypical plasma-cells.


Figure 1Figure 2
Figure 1. Infiltrated violaceous mass located on perianal area
Figure 2. Dermal infiltration of atypical plasma-cells consistent with cutaneous plasmacytoma

Weekly-sessions of VAD (vincristine, adriamicine, and dexamethasone) polichemotherapy were started with partial response. After the fourth cycle of VAD, the development of a rapid-onset perianal mass was detected. Dermatological examination revealed a firmly indurated and infiltrated erythematous-to-purple plaque involving perianal area (Fig. 1). Skin biopsy showed a dense infiltration of atypical plasma-cells involving the whole dermis and subcutaneous tissue (Fig. 2). Immunohistologically, a population of CD38+, CD138+, lambda+, and D1-cycline negative plasma-cells was seen. Based on clinical and histological findings, a diagnosis of MCP was made.


Discussion

Skin manifestations of MM may be divided into specific and nonspecific lesions. Specific skin manifestations include EP of skin and mucous membrane and tumors of the skin that are extensions of bone tumor. Nonspecific manifestations include a cutaneous finding owing to the abnormal level of proteins (amyloid, cryoglobulins), cytopenia, and involvement of internal organs (kidney, lungs, nerves, and ganglion). Other cutaneous manifestations of MM have been described, including pyoderma gangrenosum, leukocytoclastic vasculitis, Sweet syndrome, subcorneal pustular dermatosis, plane xanthoma, scleromyxedema, Waldenström macroglobulinemia cutis, amyloidosis, and cryoglobulinemias [3].

Extramedullary plasmacytomas are unusual plasma-cell tumors that arise outside the bone marrow. Although mostly seen in upper respiratory and gastrointestinal tract, EMPs have also been described in many other sites such as genito-urinary tract, skin, lung, kidney, bladder, breast, thyroid, lymph nodes and others [4]. Extramedullary plasmacytomas of the skin are extremely rare and they can be divided into primary cutaneous plasmacytoma (PCP) and 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 tumor or, more frequently, by direct extension from underlying bone lesions. Metastatic cutaneous plasmacytomas usually consist of erythematous nodules or plaques dome-shaped and smooth-surfaced ranging from 1 to 5cm in diameter. Chest, back, and abdomen are the most frequently involved areas followed by face, scalp, neck, and extremities [1, 3]. Unusual localizations have been described related to MCP, including scrotum [5], eyelid [6], 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 perianal area. Therefore, MCP should be considered in the differential diagnosis of perianal lesions.

References

1. Requena L, Kutzner H, Palmedo G, et al. Cutaneous involvement in multiple myeloma: a clinicopathologic, immunohistochemical, and cytogenetic study of 8 cases. Arch Dermatol 2003; 139: 475-486

2. Bayer-Garner IB, Smoller BR. The spectrum of cutaneous disease in multiple myeloma. J Am Acad Dermatol 2003; 48: 497-507

3. Yoon YH, Cho WI, Seo SJ. Case of multiple myeloma associated with extramedullary cutaneous plasmacytoma and pyoderma gangrenosum. Int J Dermatol 2006; 45: 594-597

4. Lin B, Weiss LM. Primary plasmacytoma of lymph nodes. Hum Pathol 1997; 28: 1083-1090

5. Switzer PK, Moseley V, Cannon WM. Extramedullary plasmacytoma involving pharynx, skin, and lymph nodes. AMA Arch Intern Med 1950; 86: 402-411

6. Walzer RA, Shapiro L. Multiple myeloma with cutaneous involvement. Dermatologica 1967; 134: 449-454

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