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| CASE REPORT |
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| Year : 2015 | Volume
: 42
| Issue : 1 | Page : 45-47 |
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Cytological diagnosis of solitary plasmacytoma of the skull: A rare case report
Mohamad Banyameen Iqbal1, Iqra Mushtaq2, Tushar Kambale1, Atul Jain1
1 Department of Pathology, Dr. D.Y. Patil Medical College, Hospital and Research Center, Pimpri, Pune, Maharashtra, India
2 Department of Ophthalmology, Dr. D.Y. Patil Medical College, Hospital and Research Center, Pimpri, Pune, Maharashtra, India
| Date of Web Publication | 16-Jan-2015 |
Correspondence Address:
Mohamad Banyameen Iqbal
Department of Pathology, Dr. D.Y. Patil Medical College and Hospital, Pimpri, Pune, Maharashtra
India
 Source of Support: None, Conflict of Interest: None  | Check |
DOI: 10.4103/0974-5009.149486
Solitary plasmacytoma (SPC) of the skull (SPS) is rare, and only a few cases have been reported in the literature so far. Plasmacytoma of the skull has a wide spectrum of pathology, including a quite benign, SPC, and an extremely malignant, multiple myeloma at the two ends of the spectrum. SPC of bone including SPS is characterized by a radiologically solitary bone lesion, neoplastic plasma cells in the biopsy specimen, fewer than 5% plasma cells in bone marrow, <2.0 g/dl monoclonal protein in the serum when present and negative urine test for Bence Jones protein (monoclonal light chain). For diagnosing, a comprehensive examination and analysis, which includes radiological examination, immunoglobulin, biochemistry, test for Bence Jones protein in the urine and bone marrow is needed. Keywords: Multiple myeloma, plasmacytoma, solitary plasmacytoma
How to cite this article:
Iqbal MB, Mushtaq I, Kambale T, Jain A. Cytological diagnosis of solitary plasmacytoma of the skull: A rare case report. J Sci Soc 2015;42:45-7 |
| Introduction | |  |
Pathological proliferation of the plasma cell population produces a wide spectrum of disorders, ranging from benign solitary plasmacytoma (SPC) to malignant multiple myeloma (MM). [1]
True SPC of the skull (SPS) without signs of systemic myelomatosis is very rare. SPC of bone including SPS is thought to be a kind of monoclonal gammopathy comprising 3-5% of patients. [2] The appearance of a single osteolytic plasmacytoma of the skull without signs of systemic myelomatosis is extremely rare. [3],[4] The prognosis for SPC of the cranial vault appears to be good when it is diagnosed on strict criteria, which is based on a radiologically solitary bone lesion, neoplastic plasma cells in the biopsy specimen, <5% plasma cells in bone marrow, <2.0 g/dl monoclonal protein in the serum when present and negative urine test for Bence Jones protein (monoclonal light chain). [5]
| Case report | | |
A 65-year-old female first noted a painless, soft swelling mass, 8 cm × 8 cm in diameter, in the parieto-occipital region. Neurological examination identified a relatively fixed mass with no tenderness and no abnormalities. Computed tomography (CT) showed a large extradural mass [Figure 1] with homogeneous enhancement after intravenous administration of contrast material and bone CT revealed a solitary osteolytic lesion involving the whole layer of the skull. Magnetic resonance imaging showed that the mass was mostly isointense with the brain parenchyma on both T1- and T2-weighted images was homogeneously enhanced. Laboratory examinations showed a red blood cell (RBC) count of 3.90 × 10 12 /l; hemoglobin, 9.9 g/l; white blood cell count, 5.2 × 10 9 /l; neutrophils, 54.5%; lymphocytes, 34.5%, which were all within the normal range. A urine test for Bence Jones protein was negative. Renal function showed abnormal levels of blood urea nitrogen 10.32 mmol/l and creatinine 220.6 mol/l. Bone marrow examination was done in this patient, which showed normal results, especially of plasma cells. | Figure 1: Computed tomography scan showing a lesion at the parietal region
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Microscopy
Multiple smears were taken from the lesion and stained with leishman and hematoxylin and eosin stains and were examined under microscope revealing multiple plasma cells with characteristic eccentrically placed nucleus and a prominent perinuclear hof, these plasma cells were seen in a background of RBC's, which were showing rouleaux formations all through the smears [Figure 2] and [Figure 3]. | Figure 2: Fine needle aspiration cytology showing large collections of atypical plasma cells and red blood cells in rouleaux formation (Leishman, ×100)
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 | Figure 3: High power view showing atypical plasma cells (Leishman, ×200)
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| Discussion | | |
Myeloma is a malignant tumor, which originates from the reticulocytes of the bone marrow. The tumor cells have the characteristics of plasmacytes, so the disease was known as plasma cell myeloma. [3] Plasma cell myeloma mostly occurs in the elderly over 40 years of age; the median age of individuals with the disease in the United States was 62 years old. [3] Only 2-3% of patients with the disease are younger than 30 years old. [4] Bone destruction due to myeloma may occur in any area of the body. Its incidence is as follows: Spine, 49%; skull, 35%; pelvis, 34%; ribs, 33%; humerus, 22%; femur, 13%; mandible, 10%. [3] Tumors which occur in the skull are called cranial myelomas and are also known as cranial plasma cell tumors. Single tumors are rarely seen in the clinic. [4] The diagnosis of skull myeloma also needs to be differentiated from eosinophilic granuloma, osteosarcoma, and metastatic carcinoma. [4] Eosinophilic granuloma is mainly observed in children and young individuals, and most occur singly (85%), which aids the identification of skull myeloma. Eosinophilic granuloma may mostly exhibit bone lesions or a large number of eosinophilic granulomas. The skull is the predilection site, with tumors often located in the frontal, temporal, and parietal bones. Tumors have a rich blood supply but have clear boundaries from the surrounding tissues. The size of eosinophilic granuloma is usually small, rarely more than 2-3 cm. The edge of the tumor often has a "clivus-like" or "bilateral-like" appearance, and a sequestrum of the "button" type may be observed. Osteosarcoma is a common primary malignant bone tumor, which is mostly found in long bones, with few cases in the skull. Osteosarcoma may be divided into osteolytic type, bone, and mixed type. The osteolytic type mainly shows bone destruction with a round or irregular shape, with blurred contours. The bone destruction mainly occurs in the outer cranial plate and large extracranial soft tissue is often observed, in which the bone tumor may be found. Osteolytic metastasis is the most common type of metastasis, and the metastases are often multiple and of a range of sizes, with osteolytic bone destruction. The edges of metastases may be blurred and there may be an associated small adjacent soft tissue mass. A small number of osteolytic metastases show single bone destruction with a larger soft tissue mass in which residual bone chips may be observed. Naganuma et al. suggested that laboratory examination should include bone marrow examination, serum protein electrophoresis, serum immunoglobulins, blood, urine Bence Jones protein, and kidney function. [6] The International Myeloma Working Group proposed new criteria for the diagnosis and classification of myeloma based on routinely available examinations. According to the criteria, symptomatic myeloma requires evidence of an M-protein in the serum and urine, bone marrow plasmacytosis and related end-organ damage. [7] The criteria for asymptomatic, or smoldering, myeloma are M-protein levels ≥30 g/l and/or bone marrow clonal cells ≥10%, but no related organ or tissue impairment (ROTI; end-organ damage). Cases with ROTI typically present with increased calcium levels, renal insufficiency, anemia or bone lesions, which are attributed to the proliferation of plasma cells. Symptomatic myeloma requires evidence of ROTI. SPC of bone, extramedullary plasmacytoma, and multiple SPCs (± recurrent) are also defined as distinct entities. The use of these criteria should facilitate the comparison of therapeutic trial data. [8] Prior to 2011, there were only 100's of cases of SPC reported in the English literature. [9] In cases with no lesions in other parts of the body, the patients have good prognosis following surgical resection and radiotherapy. Chemotherapy is being increasingly used in the treatment of plasma cell myeloma, but radiotherapy is being used less. The prognosis of MM is not as good as SPC. [10] Dong et al. have reported a similar case report of two cases presenting with a swelling in the parietal region in which all other criteria like bence jones protein and bone marrow examination were normal [11] just as in our case. A similar case report of a 78-year-old female suffering from SPC of the cranial vault have also been reported by Zigouris et al. [12]
The patient underwent surgery (craniotomy) and the diagnosis was confirmed on histopathological examination.
| Conclusion | | |
The characteristics of myeloma are complicated. Plasmacytoma of the skull has a wide spectrum of pathology, including a quite benign, SPC, and an extremely malignant, MM at the two ends of the spectrum. The clinical features are complex and not easily identified, leading to the high misdiagnosis rate. A comprehensive examination and analysis is needed for correct diagnosis, which includes immunoglobulin, biochemistry, urine Bence Jones protein, and bone marrow.
| References | | |
| 1. | Joshi A, Jiang D, Singh P, Moffat D. Skull base presentation of multiple myeloma. Ear Nose Throat J 2011;90:E6-9.  |
| 2. | Kyle RA. Monoclonal gammopathy of undetermined significance and solitary plasmacytoma. Implications for progression to overt multiple myeloma. Hematol Oncol Clin North Am 1997;11:71-87. |
| 3. | George ED, Sadovsky R. Multiple myeloma: Recognition and management. Am Fam Physician 1999;59:1885-94. |
| 4. | Wein RO, Popat SR, Doerr TD, Dutcher PO. Plasma cell tumors of the skull base: Four case reports and literature review. Skull Base 2002;12:77-86. |
| 5. | Tanaka M, Shibui S, Nomura K, Nakanishi Y. Solitary plasmacytoma of the skull: A case report. Jpn J Clin Oncol 1998;28:626-30. |
| 6. | Naganuma H, Sakatsume S, Sugita M, Satoh E, Asahara T, Nukui H. Solitary plasmacytoma of the skull: Immunohistochemical study of angiogenic factors and syndecan-1 - Two case reports. Neurol Med Chir (Tokyo) 2004;44:195-200. |
| 7. | Hotta T. Classification, staging and prognostic indices for multiple myeloma. Nihon Rinsho 2007;65:2161-6. |
| 8. | International Myeloma Working Group. Criteria for the classification of monoclonal gammopathies, multiple myeloma and related disorders: A report of the International Myeloma Working Group. Br J Haematol 2003;121: 749-57. |
| 9. | Lorsbach RB, Hsi ED, Dogan A, Fend F. Plasma cell myeloma and related neoplasms. Am J Clin Pathol 2011;136:168-82. |
| 10. | Kyle RA, Therneau TM, Rajkumar SV, Larson DR, Plevak MF, Melton LJ 3rd. Incidence of multiple myeloma in Olmsted County, Minnesota: Trend over 6 decades. Cancer 2004;101:2667-74. |
| 11. | Dong L, Zhang X, Zhang H, Song R, Gu X, He L. Solitary plasmacytoma of the skull: Two case reports. Oncol Lett 2013;5:479-82. |
| 12. | Zigouris A, Drosos D, Alexiou GA, Fotakopoulos G, Mihos E, PahatouridisD, et al. Primary plasmacytoma of the cranial vault: a case report. Cases J 2009 7;2:9154. |
[Figure 1], [Figure 2], [Figure 3]
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