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Objective: In order to better understand the condition and provide the groundwork for early detection and treatment of plasmacytomas, it is important to examine the clinical characteristics, therapeutic options, and effectiveness of plasmacytomas that are initially identified with head and neck symptoms. Methods: Retrospective analysis, evaluation, and discussion of the clinical data of 7 patients with plasmacytoma initially diagnosed with head and neck symptoms and admitted to the Affiliated Hospital of Qingdao University during the period of June 2013 to November 2022 was done in combination with pertinent literature. Results: All seven patients were diagnosed with plasmacytoma by histopathology, with lesions located in the nasopharyngeal oropharynx in 4 cases, nasal sinuses in 2 cases, and ventricular zone in 1 case. Clinical manifestations and imaging were atypical, with localized manifestations, of which 2 cases were accompanied by multiple skeletal lesions throughout the body, and 4 cases had lymph node metastasis. Surgery was preferred for all patients, and individualized treatment was recommended after surgery. Of the 7 patients, 3 patients underwent surgery and chemotherapy, 2 patients underwent surgery and radiotherapy and chemotherapy, 1 patient underwent surgery and radiotherapy, and 1 case was treated with surgery only. The follow-up period was 3-60 months, with a 100% follow-up rate. 5 cases were alive and 2 cases died of multiple myeloma after 4-5 years Conclusion: Plasmacytomas first diagnosed with head and neck symptoms are rare, and extramedullary plasmacytomas have a better prognosis, while more advanced multiple myeloma has a poorer prognosis; Therefore, enhancing the quality of survival as well as the duration of survival for patients with plasmacytomas requires early diagnosis and individualized treatment., Competing Interests: The authors of this article and the planning committee members and staff have no relevant financial relationships with commercial interests to disclose., (Copyright© by the Editorial Department of Journal of Clinical Otorhinolaryngology Head and Neck Surgery.)

Extramedullary plasmacytoma (EMP) is a special type of malignant plasmacytosis, which is complex and heterogeneous. Most EMP patients have poor prognosis and lack a stratified prognostic system or ideal treatment strategy supported by evidence-based medical evidence, which cannot meet clinical needs. In order to improve the understanding of this disease entity, Plasma Cell Disease Group, Chinese Society of Hematology, Chinese Medical Association and Chinese Myeloma Committee-Chinese Hematology Association developed the "Chinese Expert Consensus on the diagnosis and treatment of extramedullary plasmacytoma", which aims to standardize the clinical diagnosis and treatment of EMP and ultimately improve the overall survival of patients with plasmacytoma.

Objective: To explore the clinical characteristics, treatment methods and outcomes of extramedullary plasmacytoma of the head and neck. Methods: A retrospective analysis was conducted on 10 cases with extramedullary plasmacytoma of the head and neck who were admitted to Henan Tumor Hospital from January 2005 to January 2020. Among the 10 patients, 6 were male and 4 were female. The average age at diagnosis was 56.3 years old (34-74 years old). Among them, 3 cases were located in the nasal cavity, 2 cases in the nasopharynx, 1 case in the sinuses, 2 cases in the larynx, 1 case in the oropharynx, and 1 case in the cervical lymph nodes. Treatments were administered according to tumor size and resection extent. Complete surgical excision (negative margins) was preferred, followed by adjuvant radiotherapy or radiotherapy alone. The clinical characteristics, diagnosis, treatment and prognosis of EMP were analyzed. Results: The patients' symptoms were not specific, frequently with local obstruction symptom and localized masses. All patients were confirmed pathologically as suffering from monoclonal plasmacytoma, with negative bone marrow biopsy and negative skeletal survey. Five patients received surgery, 3 received radiotherapy, and 2 received surgery with additional radiation. The follow-up time was 16-125 months, with a median of 92 months. Two patients developed into multiple myeloma. One patient who received radiotherapy after surgery relapsed after 7 years of follow-up and again received surgical treatment, with no evidence of second recurrence. The remaining patients had no recurrence or progression. Conclusion: Extramedullary plasmacytoma of the head and neck has a good prognosis. Surgical treatment can be considered for completely resectable lesions.

A collection of plasma cells in the skin can represent a broad spectrum of disease entities. Secondary syphilis, primary cutaneous plasmacytoma, primary cutaneous plasmacytosis, cutaneous lymphoid hyperplasia and nodular amyloidosis are considered possible differential diagnoses. The primary cutaneous plasma cell disorders can range from malignant to benign plasma cell neoplasms. The malignant conditions are neoplastic diseases having monoclonal proliferations, rapid progression and fatal outcome while the benign plasma cell disorders usually show polyclonality, chronicity and benign process, including plasmacytosis. We present a case of cutaneous plasmacytosis. The patient was a 34-year-old man, presented with disseminated reddish-brown plaques and nodules on the right side of the hips, inguinal groove, and the thigh. Histopathologically, mature plasma cells perivascular infiltrates were observed mainly in the dermis. Polyclonality of infiltrating plasma cells with coexistence of both kappa and gamma chain-positive cells demonstrated with immunohistochemistry, as well as CD20+++, CD38++++, CD79a++++, CD138++, Ki67<30%. The diagnosis, cutaneous plasmacytosis, was established by the pertinent laboratory findings. Primary cutaneous plasmacytosis was an uncommon reactive lymphoplasmacytic disorder of uncertain etiology. Cutaneous plasmacytosis is a rare disease characterized by peculiar multiple eruptions and hyper gamma globulinemia. It has been mainly described in patients of Japanese descent, with only few reports in Caucasians and Chinese, although information concerning the disorder was limited to individual case reports. Cutaneous plasmacytosis is a rare disorder, which is characterized by multiple red to dark-brown nodules and plaques on the trunk and usually associated with polyclonal hyper gamma globulinaemia. Primary cutaneous plasmacytosis or cutaneous plasmacytosis was thought to be a reactive process with unknown etiology. Histologically, lesions contain dense perivascular infiltration of mature polyclonal plasma cells without any atypia, in the dermis and subcutaneous fat. The clinical course is chronic and benign without spontaneous remission. Available treatments for cutaneous plasmacytosis include psoralen ultraviolet A radiotherapy, systemic chemotherapy and intralesional steroid injection. The patient with cutaneous plasmacytosis in this report was treated with tacrolimus ointment and psoralen ultraviolet A.

Objective: To investigate the clinical manifestation, treatment and prognosis of extramedullary plasmacytoma(EMP) in the upper airway, and to improve the diagnosis and outcome of EMP treatment., Method: Clinical data of 26 EMP cases were reviewed retrospectively, and then compared with multiple myeloma(MM) patients presenting with lesions in upper airway., Result: Of 26 cases, 9 cases with the tumors occurred in nasal cavities, 7 in nasal sinuses, 6 in pharyngeal, 4 in throat, mainly manifesting with local masses and relevant symptoms. The manifestations of clinical, endoscopy findings and pathologic results in EMP patients were not distinguishable from the lesions of MM patients, while MM patients often accompanied by other findings, such as anemia and bone damage. Involvement of neck lymph nodes was more common in MM patients than in EMP patients. Ten patients were treated with surgery, and 16 patients with surgery and radiotherapy. Of the seven EMP patients with involvement of neck lymph nodes, four patients received additional chemotherapy besides surgery and radiotherapy, and no local relapse and MM happened in them, while of the three patients only received surgery and radiotherapy, one local relapse were found and one progressed to MM., Conclusion: The diagnosis of EMPs mainly depends on pathological results. The judgment of pathologists and application of molecular biology technology are vital for the diagnosis of EMP in upper airway, and MM must be excluded very carefully in the diagnosis of EMP. Surgery combined with radiotherapy is the main treatment for EMP in the upper airway, and the prognosis is good but the follow-up should be taken. Besides surgery and radiotherapy, chemotherapy is beneficial for the EMP patients accompanied with lesions in neck lymph nodes.

Objective: To compare the pathologic diagnosis and immunohistochemistry of small cell malignant tumors (SCMT) of bone using both core needle biopsy and surgical specimen., Methods: Seventy-seven cases of SCMT with core needle biopsies and surgical specimens available were respectively analyzed by histologic examination and immunohistochemical study, with literature review., Results: The male-to-female ratio was 48:29. The age of the patients ranged from 6 to 73 years. The tumors studied included Ewing sarcoma/PNET (n = 38), myeloma (n = 23), lymphoma (n = 10), small cell osteosarcoma (n = 2), small cell carcinoma (n = 2) and mesenchymal chondrosarcoma (n = 2). The tumors involved limbs, axial skeleton and flat bones. Microscopically, the tumors shared similar histology, with small round cells and spindly cells arranged in diffuse sheets. The pathologic diagnosis by core needle biopsies correlated with that by surgical specimens in 84.4% (65/77) of the cases., Conclusions: SCMT represents a heterogeneous group of malignancy. Correlations with clinicoradiologic findings and application of ancillary investigations including immunohistochemistry and molecular study are important for definitive diagnosis. Pathologic diagnosis using core needle biopsies shows good results and provides useful information for surgical planning.

Patient of plasmacytoma of temporal bone often suffered from evident symptom in unilateral ear and obvious damage of bone. Clinical characteristics, imaging examination and histopathology can established an accurate diagnose. The therapy will be surgery and followed radiotherapy.

Objective: To explore the clinical features, treatment and prognosis of multiple myeloma (MM) with extramedullary plasmacytomas (EM)., Methods: A total of 43 patients were enrolled and divided into 2 groups. Group-1 had 12 patients of EM occurring after the diagnosis of MM while Group-2 included 31 EM patients at the initial diagnosis of MM., Results: The male-to-female proportion was 23:20 and there was a median age of 53 years. The distribution of different isotypes was IgG (n = 15), IgA (n = 9), IgD (n = 2), κ light chain (n = 6), λ light chain (n = 6), biclonal myeloma (n = 3) and nonsecretory myeloma (n = 2). The sites of complicating plasmacytoma included skin, muscle and spinal canal. Nine patients received bortezomib plus DECP (cisplatin, etoposide, cyclophosphamide and prednisone) and 2 patients underwent traditional chemotherapy in Group-1. The outcomes were as follows: complete remission (CR, n = 2), partial remission (PR, n = 4) and death (n = 5). And 11 patients received traditional chemotherapy in Group-2, 7 attained PR and 4 died. Twenty patients received bortezomib plus other chemotherapeutic drugs in Group-2. The outcomes were as follows: CR (n = 12), PR (n = 7) and death (n = 1). The median overall survival (OS) was 36 months (range: 10 - 120) in Group-1 and 23 months (range: 5 - 52) in Group-2 respectively. In Group-1, the estimated 3- and 5-year OS were 54.21% and 27.10% respectively. And in Group-2, the estimated 3- and 4-year OS were 39.83% and 13.28% respectively., Conclusions: The EM patients show aggressive, complicated and diverse clinical courses and the unusual manifestation of multiple organ involvement by plasma cells. Traditional chemotherapy has a poor efficacy and the prognosis is unfavorable, especially for EM with concurrent MM. The combined treatment of bortezomib with second-line chemotherapy may achieve curative effects.

Objective: To study the diagnostic accuracy of hematolymphoid malignancy by using effusion fluid cytology specimens and to evaluate the values of immunocytochemistry for this assay., Methods: The cytospin preparations/smears and cell block sections of effusion cytology specimens from 33 cases of hematolymphoid malignancy were retrospectively reviewed. Immunocytochemical study was performed. In selected cases, in-situ hybridization for Epstein-Barr virus-encoded RNA and immunoglobulin and T-cell receptor gene rearrangement study were carried out as indicated., Results: There were 33 cases of hematolymphoid malignancy, including 12 cases of T-lymphoblastic leukemia/lymphoma, 16 cases of mature B cell neoplasm (including 9 cases of diffuse large B-cell lymphoma, 2 cases of Burkitt lymphoma, 2 cases of plasmacytoma/multiple myeloma, 2 cases of B-small lymphocytic leukemia/lymphoma and 1 case of mantle cell lymphoma), 3 cases of mature T or NK-cell neoplasm (including 1 case of extranodal nasal NK/T-cell lymphoma, 1 case of angioimmunoblastic T-cell lymphoma and 1 case of T-cell prolymphocytic leukemia), 1 case of myeloid sarcoma and 1 case of mast cell sarcoma. Amongst the 33 cases studied, 16 represented disease relapses, including 8 cases of diffuse large B-cell lymphoma, 2 cases of plasmacytoma/multiple myeloma, 2 cases of B-small lymphocytic leukemia/lymphoma, 1 case of T-lymphoblastic leukemia/lymphoma, 1 case of angioimmunoblastic T-cell lymphoma, 1 case of mantle cell lymphoma and 1 case of mast cell sarcoma. The remaining 17 cases showed serous effusion as the primary manifestation, with the diagnosis primarily made upon cytologic examination. The cytologic findings seen in all the 33 cases studied were in agreement with the corresponding histologic diagnosis., Conclusions: Diagnosis of hematolymphoid malignancy by effusion fluid cytology specimens is possible, especially when coupled with the clinical history, immunophenotype, in-situ hybridization and gene rearrangement study findings. This is especially so for cases with disease relapses.

Objective: To study the clinical, radiologic and pathologic features of solitary plasmacytoma of spine., Methods: The clinical, radiologic and pathologic features, as well as treatment and follow-up data, of 13 solitary plasmacytoma of spine cases were retrieved and analyzed. Immunohistochemical study using EnVision method for LCA, CD19, CD20, CD79a, CD3, CD7, PC, MUM1, CD138, IgG, IgM, kappa, lambda and Ki-67 was carried out., Results: All the tumours were primarily located in the vertebrae (including 9 cases in thoracic vertebrae and 4 cases in lumbar vertebrae). The male-to-female ratio was 3.3:1. The age of the patients ranged from 42 to 69 years (mean age = 56 years). The commonest symptom was pain in the surrounding regions. The degree of neurologic disturbance mostly depended on the extent of vertebral destruction and structural instability of the spine. Radiologic examination showed mainly osteolytic lesions in vertebrae. Magnetic resonance imaging demonstrated the presence of heterogeneous intensity inside the involved vertebrae (low in T1 weighted and high in T2 weighted images). Histologic examination showed diffuse infiltration by malignant cells. In well-differentiated plasmacytomas, the tumor cells resembled normal plasma cells. In poorly differentiated examples, the cellular morphology mimicked that of the centroblasts. The interstitial stroma was scanty and contained plenty of vessels, sometimes with formation of blood lakes. Amyloid deposition was present in some of the cases. Immunohistochemical study showed that the tumor cells were positive for CD79a and negative for CD20. Light chain restriction was detected in all the 13 cases studied. Plasma cell marker PC was expressed in all cases, while IgG was positive in 5 cases, IgM in 1 case, MUM1 in 10 cases and CD138 in 8 cases. Ki-67 index varied from 10% to 50%. All cases were operated, with adjuvant chemotherapy and radiotherapy given., Conclusions: Correlation of clinical, radiologic and pathologic features is important in diagnosis of solitary plasmacytoma of spine. The possibility of multiple myeloma needs to be excluded. Early detection by radiologic examination, local surgical resection, post-operative chemoradiotherapy and long-term follow-up are prudent for successful management of this condition.

Objective: To summarize the clinical features and the experiences of diagnosis and treatment of extramedullary plasmacytomas (EMPs), and to enhance the cure rates of EMPs., Method: Clinical data of 8 patients with EMPs in head and neck, who treated in our hospital from Jan. 1990 to Dec. 2004, were reviewed. Of 8 cases, 3 cases with the tumors occurred in nasal cavities, 1 in maxillary sinus, 2 in nasopharynx, 1 in posterior wall of oropharynx., Result: Eight patients who were all pathologic confirmed EMP accepted surgical resections of the tumors, and 4 of 8 cases were boosted radiation therapy post-operation. Four cases were still alive disease-free for more than 1, 5, 10 and 12 years after treatments, respectively. One died of local recurrence in 2 years, 1 died of multiple myeloma in 3 year, and 1 died of heart attack in 2 years after treatments, respectively. One lost follow-up., Conclusion: EMPs in head and neck are low potential malignancy tumors. The diagnosis of EMPs mainly depends on clinical manifestations and pathological results. Surgery and radiation therapy are the main treatments for EMPs in head and neck.

Background & Objective: Extramedullary plasmacytoma (EMP), a rare tumor, often appears in the head and neck region. About 80% of EMPs occur in the submucosa of the upper aerodigestive tract. On both clinical presentation and pathologic examination, these tumors may be confused with some common tumors in the head and neck. This study was to evaluate clinical feature, diagnosis, and treatment of EMP in the head and neck., Methods: Clinical data of 10 consecutive patients with pathologically confirmed EMP in the head and neck, treated in our hospital from Jan. 1977 to Dec. 2003, were reviewed. Of the 10 patients, 2 underwent surgery alone, 2 underwent surgery plus adjuvant radiotherapy,4 received radical radiotherapy, 1 received radical radiotherapy plus adjuvant chemotherapy, and 1 gave up treatment., Results: The patients mainly manifested as local masses and relevant symptoms. Of the 3 patients received immunohistochemical staining to detect IgG, 1 was lambda type, and 2 were kappa type. Six patients received Bence-Jones protein detection, and 4 patients received serum myeloma protein detection; all of them showed negative results. Bone marrow biopsy was made in 4 patients, skeletal survey was made in 4 patients; all results were normal. By the end of follow-up (Mar. 2003), 6 patients were still alive (5 patients survived for more than 1 year, 3 of the 5 patients survived disease-freely for more than 15 years); 1 died of local recurrence 2 years after diagnosis; 1 died of multiple myeloma; 1 died of heart disease before treatment; 1 lost follow-up., Conclusions: The diagnosis of EMP mainly depends on clinical manifestation and pathologic results. Radiotherapy and surgery are the main treatments for EMP in the head and neck.

Among plasma cell disorders, solitary plasmacytoma (solitary plasmacytoma of bone, SPB and extramedullary plasmacytoma, EMP) is rare as compared with multiple myeloma (MM). Furthermore, the relationship between solitary plasmacytoma and MM remains unclear. Between 1960 and 1994, 24 patients with SPB and 20 with EMP were treated. The criteria for diagnosis were: (1) no evidence of other lesions based on clinical and radiologic examinations; (2) biopsy evidence of a plasma cell neoplasm; (3) bone marrow biopsy specimen with negative findings (less than 10% plasma cells); (4) no anemia, hypercalcemia or renal involvement. The average follow-up period was 112 months (from 6 to 360 months). Fifty-four percent of patients with SBP and 40% of patients with EMP developed MM, however, there was no significant statistical difference between SPB and EMP (P > 0.05). We suggested that solitary plasmacytomas be classified as two types, latent and aggressive. The former was histologically well-differentiated plasmacytoma. The latter was poorly differentiated tumors which easily progress to MM. The treatment of choice is wide excision or thorough curettage, by cryogenic necrosis with liquid nitrogen or cautery of the bony wall with phenol and the cavity filled with bone grafts or cementation. All patients with apparently isolated plasmacytoma should receive local radiotherapy after operation. Adjuvant chemotherapy should be given if the tumour turns out to be poorly differentiated, in order to delay their progression to MM.

Plasma cell neoplasm have been classified as multiple myeloma, solitary plasmacytoma and extramedullary plasmacytoma. The solitary plasmacytoma of maxilla is a rare condition. It is a single focus of myelomatous tissue with no dissemination to other parts of the skeleton. This paper presents a case of solitary plasmacytoma in the maxillary bone. Roentgenographic examination revealed a cystic-like osteolytic lesion over the left posterior portion of maxillary bone, invading maxillary sinus. The CT scan showed the tumor mass occupied the left maxillary sinus and lateral wall of the nasal cavity, protruding to the buccal side of the left face. The tumor cells were composed of densely packed round and polygonal cell structures which were scattered in relatively sparse stoma. The neoplastic cells have large, single eccentric nucleus, resembling typical plasma cells. The ultrastructural studies of tumor cells revealed numerous endoplasmic reticulum. There were arranged in a lamellar pattern, large numbers of mitochondria in perinuclear distribution and prominent Golgi complex. Nuclei showed patch condensation of chromatin and large nucleoli. The monoclonal staining (anti-Kappa and anti-IgG) of tumor cells by the peroxidase anti-peroxidase immunohistochemical technique, proved that the plasma cell lesion is neoplastic in nature. The tumor mass was eradicated by total resection, followed by X-ray radiation (4200 RADS). The patient is in good physical condition. There has been no clinical evidence of recurrence three years after surgery.

A 49 year old man had had headache and some cranial nerve paralyses for about three years. A tentative diagnosis of meningioma of the skull base or chordoma was made before his death. In the postmortem examination, a large tumor lying on the skull base was found. The sella turcica, a part of the basilar bone were destroyed. The neoplasm had invaded the adjacent cerebrum, cranial nerves and extra-cranial tissues. Histopathologically, the lesion showed a typical poorly-differentiated plasmacytoma. Both clinic and pathologic characteristics of this tumor are discussed and the literature on solitary intracranial plasmacytoma is reviewed.

Extramedullary plasmacytoma (EMP), being different from multiple myeloma, is very rare. One case of primary EMP of small intestine and review of literature are presented. The patient, 69-year-old male, was admitted because of left lower abdominal pain and anemia. A mass, table-tennis in size, was palpated. Intestinal obstruction was found by plain X-ray film. It was diagnosed as EMP of small intestine by pathology after exploratory celiotomy. Immunohistochemical stain showed that intracytoplasmic immunoglobulin was monoclonal IgG. Lambda. The blood Bence-Jones protein and bone marrow puncture were negative. The patient received chemotherapy after operation and was followed for two years giving satisfactory result. The pathogenesis, clinical feature, diagnostic criteria and treatment are discussed.

13 patients with extra-medullary plasma cell tumor treated by radiotherapy in a Cancer Hospital from March 1958 to December 1983 are reported. The tumor occurred more frequently in the head and neck, particularly in the upper respiratory tract and oral cavity. Grossly, the majority of the tumor showed dark reddish polypoid masses with the surface bled easily. A misdiagnosis of polyp or hemangioma was usually made. The tumor is sensitive to radiotherapy. In the patients with recurrence or metastasis, retreatment is still effective, at least, palliative. The 5 year survival rate was 70%. A dose of 4,000-6,000 rad in 4-6 weeks is advised for the primary tumor.