Open Access

Extramedullary plasmacytoma of the larynx: Literature review and report of a case who subsequently developed acute myeloid leukemia

  • Authors:
    • Shenglei Ge
    • Ganghua Zhu
    • Yan Yi
  • View Affiliations

  • Published online on: June 19, 2018     https://doi.org/10.3892/ol.2018.8992
  • Pages: 2995-3004
  • Copyright: © Ge et al. This is an open access article distributed under the terms of Creative Commons Attribution License.

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Abstract

Extramedullary plasmacytoma (EMP) of the larynx is an extremely rare plasma cell neoplasm outside of the bone marrow, which has not been previously well characterized. A case of laryngeal EMP who developed acute myeloid leukemia (AML) following treatment is described in the present study, as well as an extensive review of the relevant literature. An electronic literature search was performed in PubMed and all pertinent case reports and series in the English language from 1948‑October 2017 were identified. A total of 99 cases including the present case were available for review. The mean age of the included patients was 53 years. Supraglottis was the most frequently involved site. The most common treatment modality was radiotherapy alone (n=41; 43%), followed by a combination of surgery and radiotherapy, then surgery alone. However, for cases published in recent years, the most common treatment modality was surgically based treatment. Overall the treatment outcome was favorable, as a total of 84% of patients were alive after a mean follow‑up of 60 months. However, EMP outcomes for patients with cervical lymphadenopathy or multiple sites involvement were unfavorable with >40% of patients relapsing or developing metastasis during the limited follow‑up period. A total of 6 subjects developed multiple myeloma and 1 patient converted to AML. The present study provides important insights on the treatment of EMP, which is a rare disease. To the best of our knowledge, this is the first case report of a patient with laryngeal EMP who developed AML following treatment. It is recommended that secondary myeloid neoplasm should be considered besides multiple myeloma during the follow‑up period.

Introduction

Extramedullary plasmacytoma (EMP) is rare, accounting for approximately 3% of all plasma cell neoplasms. Up to 80% of EMP cases occur in the head and neck region, particularly in the upper aerodigestive tract, which constitutes less than 1% of head and neck tumors (1,2). Most head and neck EMPs occur in the sinonasal region, and fewer are found in the larynx (3,4). Although the primary cause of mortality is actually progression to multiple myeloma (MM), conversion to MM is uncommon (11–30% incidence). Here, we describe an individual with laryngeal EMP who developed acute myeloid leukemia (AML), rather than MM. Due to the rarity of this tumor, most previous studies focused on a case or case series. In an effort to describe this rare tumor accurately, we made a literature review about its clinical features, diagnosis, treatment modalities, outcomes, and potential sequelae of this disease.

Materials and methods

Literature review

An electronic literature search was performed in PubMed using the following terms ‘plasmacytoma’, ‘extramedullary plasmacytoma’, ‘plasma cell tumor’ in combination with the terms ‘larynx or head and neck’. Articles published from January 1948 to October 2017 were reviewed to identify cases of laryngeal EMP. Nonhuman, duplicates, and non-English language research were excluded. Abstracts were first reviewed to screen articles that discussed cases of laryngeal EMP, and then full-text articles were reviewed for extraction of data. References of the included studies were also examined for additional cases. Individual patient data were collected on age, sex, presentation, site of lesions, treatment course, long-term follow-up and outcomes. Meanwhile, articles for which individual patient data was not available or which focused solely on radiologic, histopathological findings, and diagnosis, were also excluded.

Statistical analysis

All the recorded treatment modalities are classified in two main categories: Surgically based treatment including surgical resection either alone or with adjuvant radiotherapy, and no-surgically based treatment. Differences between the above two treatment modalities were analyzed by chi-square test. SPSS version 20 statistical software (IBM Corp., Chicago, Illinois) was used, and P<0.05 was considered to indicate a statistically significant difference for all tests.

Ethics statement

This study was approved by the ethics committee of the Second Xiangya Hospital, Central South University (Changsha, China). Written informed consent was obtained from the patient.

Results

The initial database search yielded 2,022 studies

The articles that were in non-English language, animal research, or duplicate articles were excluded, and 270 studies were left. Next, those unrelated abstracts such as solely focusing on imaging examination or histopathological findings were eliminated, and a total of 127 articles were left for further analysis. The articles in which full-text was unavailable or individual data was incomplete were ruled out. The bibliographies were also examined for additional cases. Finally, 70 studies comprising a total of 98 cases were left for analysis. Therefore, a total of 99 unique patients including our case were identified and the individual patient data collected are given in Table I (571). The clinical characteristics for the 99 patients included were summarized in Table II.

Table I.

List of laryngeal EMP cases included in analysis.

Table I.

List of laryngeal EMP cases included in analysis.

Author, yearNo/sex/agePrimary sitesTreatmentLR or METFollow-upOutcome(Refs.)
The present case1/M/46Epiglottis and aryepiglottic foldRT + CTAML61 msAWD
Pino et al, 20152/M/65Left false cord and ventricleS + RTN54 msANED(5)
Wang et al, 20153/M/43Glottis, supraglottis, and subglottisSMM88 msAWD(6)
Haser et al, 20154/M/72Bilaterally vocal cords and subglottisRTN1 yANED(7)
Xing et al, 20155/F/47Left aryepiglottic foldS + RTN18 msANED(8)
Abrari et al, 20146/M/56Right vocal cordRTNNAANED(9)
Loyo et al, 20137/F/80Right glottisSNANAANED(10)
Ghatak et al, 20138/F/29True vocal cordRTN16 msANED(11)
Kim et al, 20129/M/58Left arytenoidSN2 ysANED(12)
Pinto et al, 201210/F/49Left false foldSN1 yANED(13)
Ramírez-Anguiano et al, 201211/M/57Right subglottisS + RTY1 yANED(14)
De Zoysa et al, 201212/F/62Left true vocal foldRTN2 msANED(15)
Pichi et al, 201113/M/73Left glottis and subglottisRTMM2 ysDOD(16)
Zhang et al, 201014/W/56Left false vocal cord and ventricleSN2 ysANED(17)
González Guijarro et al, 201015/M/11Right hemilarynxS + RTN3 ysANED(18)
Vanan et al, 200916/F/16Right vocal cordRTN1 yANED(19)
Pratibha et al, 200917/M/49False vocal cord, vocal cordRTN6 msANED(20)
Iseri et al, 200918/F/46Aryepiglottic foldS + RT +CTN2 ysANED(21)
Rutherford et al, 200919/F/13Subglottis, nasopharynxS + RTN6 weeksANED(22)
Ozbilen Acar et al, 200820/F/43True vocal cordSN2 ysANED(23)
Straetmans and Stokroos, 200821/M/57EpiglottisS + RTY27 msANED(1)
Velez et al, 200722/M/64Right hemilarynxS + RTN3 ysANED(24)
Kusunoki et al, 200723/F/76SupraglottisBiopsyN6 msAWD(25)
Lewis et al, 200724/M/71Supraglottis, soft palateSN2 ysANED(26)
Nakashima et al, 200625/M/39Left arytenoidS + RTN6 ysANED(27)
26/M/59EpiglottisSN15 ysANED
Sakiyama et al, 200527/F/47Subglottis, the chest wallRT + CTN7 ysANED(28)
Chao et al, 200528/M/60SupraglotisRTN37 msDOC(29)
Yavas et al, 200429/F/43Left vocal cord, nasopharynxRTNANANA(30)
Michalaki et al, 200330/F/46LarynxRTN49 msANED(31)
31/M/59LarynxRTN67 msANED
Soni et al, 200232/M/65SubglottisRTN2 ysANED(32)
Kamijo et al, 200233/M/84False vocal foldS + RTN2 yearsANED(33)
Strojan et al, 200234/M/65Left false vocal cordRTN7.8 yearsDOC(34)
Strojan et al, 200235/M/72Right true vocal cordS + RTN4.7 ysDOC
36/F/50Right glottisRTN2.2 ysANED
Nagasaka et al, 200137/F/12Right subglottisS + RTN4 ysANED(35)
Maheshwari et al, 200138/M/65SubglottisRTN12 msANED(36)
Uppal and Harrison, 200139/M/54Left hemilarynxRTMMweeksDOD(37)
Rakover et al, 200040/M/38Right true vocal foldS + RTN3 ysANED(38)
Hotz et al, 199941/NA/63Larynx, nasopharynx, nasal fossaS + RTN108 msANED(39)
42/NA/45Larynx, nasopharynxS + RTY108 msAWD
Alexiou, 199943/M/69LarynxSN62 msANED(3)
44/M/40Aryepiglottic foldS + RTN20 msANED
Nowak-Sadzikowska and Weiss,45/M/34SupraglottisRTN10 ysANED(40)
199846/M/50GlottisRTN10 ysANED
47/M/36SupraglottisRTN10 ysANED
48/F/68SupraglottisRTN10 ysANED
49/M/48GlottisRTN10 ysANED
Bhattacharya et al, 199850/F/49SupraglottisBiopsyN  6 msDOC(41)
Sulzner et al, 199851/M/49Right aryentoidRTN5 ysANED(42)
Susnerwala, 199752/F/79LarynxRTN132 msANED(2)
53/M/65LarynxRTN52 msANED
Rolins et al, 199554/M/43EpiglottisSN3 ysANED(43)
Mochimatsu et al, 199355/M/42EpiglottisS + RTMM12 ysDOD(44)
Weissman et al, 199356/M/76SubglotisS + RTNANANA(45)
Barbu et al, 199257/M/69SupraglottisRTN3 ysANED(46)
Kost et al, 199058/M/43Left vocal cordRTNANANA(47)
Gambino, 198859/M/47EpiglottisS + RTNANANA(48)
Gaffney et al, 198760/M/80LarynxRTN7 msANED(49)
Burke et al, 198661/M/53Supraglottis, and mouthCTN1 yANED(50)
Gadomski et al, 198662/F/54Bilateral true vocal cordsS + CTN15 ysDOC(51)
63/F/51Right aryeplottic foldRTN5 ysANED
Maniglia and Xue, 198364/F/64HemilarynxS + RTN1 yDOC(52)
Bjelkenkrantz et al, 198165/NARight false vocal cord, left tonsilS + RTN7 ysANED(53)
Bush et al, 198166/F/52Epiglottis, supraorbital regionRTN3 ysDOC(54)
67/F/34LarynxS + RTY5.9 ysANED
Singh, et al 197968/F/42SupraglottisS + RTN29 msANED(55)
Woodruff et al, 197969/F/64SupraglottisRTN6.5 ysDOC(56)
70/F/34SupraglottisRTNRecentlyANED
Petrovich et al, 197771/M/74EpiglottisRTN6 ysANED(57)
Gorenstein et al, 197772/M/58Right true vocal cordS + RTN3 ysANED(58)
73/M/63Right true vocal cordS + RTN25 ysANED
74/M/59SubglottisSN5 ysDOC
75/M/32SubglottisSN10 ysANED
76/M/42Bilateral true cordsSN5 ysANED
77/M/61SupraglottisRTN6 ysANED
Muller and Fisher, 197678/M/44SupraglottisBiopsyNANAAWD(59)
Fishkinand Spiegelberg, 197679/M/74Right epiglottisRTY4 ysAWD(60)
Stone and Cole, 197180/M/67Left false vocal foldRT + CTN10 msANED(61)
Poole and Marchetta, 196881/M/41Larynx, multiple sites at autopsyS + RTY3 ys 5 msDOD(62)
Webb, 196282M/62Left supraglottis, soft palateRTMM10 ysDOD(63)
83/F/55Right vocal cord and ventricleSN11 ysANED
84/M/32SubglottisS + RTN10 ysANED
Dolin and Dewar, 195685/M/74LarynxRTN3.5 ysDOC(64)
86/M/73LarynxSN1 yANED
87/M/59LarynxRTN4 ysANED
Priest, 195288/M/50Larynx, pharynx, and noseSY4 ysAWD(65)
Ewing and Foote, 195289/M/76LarynxRTN6 msAWD(66)
Costen, 195190/M/52Left epiglottisRTMM1 yAWD(67)
Rawson et al, 195091/F/59LarynxS + RTY11 ysAWD(68)
Stout and Kenney, 194992/M/46Left epiglottis, oropharynxSY14 ysANED(69)
93/F/67EpiglottisRTY6 msDOD
94/NALarynx, nasopharynx and conjunctivaSY3 ysAWD
95/M/64Larynx, nasopharynxSY2 ysAWD
96/F/48Larynx, nasopharynx, and nasal cavitySY11 ysAWD
Hodge and Wilson, 194897/M/53Left false vocal cordSN1 yANED(70)
Lumb and Prossor, 194898/M/34LarynxRTY30 msAWD(71)
99/M/20Larynx, palate, and tongueS + RTY7 ys 6 msAWD

[i] EMP, extramedullary plasmacytoma; M, male; F, female; RT, radiotherapy; S, surgery; CT, chemotherapy; LR, Local recurrence; MET, metastasis; MM, multiple myeloma; AML, acute myeloid leukemia; ys, years; ms, months; AWD, alive with disease; ANED, alive, no evidence of disease; DOD, died of disease; DOC, died of other causes; Y, yes; N, no; NA, not acquired.

Table II.

Clinical features of included cases.

Table II.

Clinical features of included cases.

Characteristics (n=95)Measure, n (% total)
Patient age, mean, median (range), years53.3, 54 (11–80)
  Male, mean (n=65)54.9
  Female, mean (n=30)50
Symptoms (n=67)
  Hoarseness46 (69)
  Dysphonia7 (10)
  Dyspnea13 (19)
  Dysphagia9 (13)
  Stridor6 (9)
  Cough6 (9)
  Sore throat3 (4)
  Hemoptysis3 (4)
  Laryngeal foreign body sensation3 (4)
Laterality (n=41)
  Right19 (46)
  Left17 (41)
  Both5 (12)
Primary site (n=79)
  Glottis19 (24)
  Supraglottis41 (52)
    Epiglottis12 (15)
    Aryepiglottic fold4 (5)
    Arytenoid3 (4)
    False vocal cord8 (10)
    Multiple sites2 (3)
    Unknown detailed site12 (15)
  Subglottis10 (13)
  Hemilarynx or 2–3 parts of the larynx9 (11)
Cervical lymph nodes involvement (n=12)
  Glottic patient1 (8)
  Supraglottic patient8 (67)
  Hemilaryngeal patient1 (8)
Coexistence with other body sites involved17
Treatment (n=96)
  Radiotherapy alone41 (43)
  Surgery alone21 (22)
  Chemotherapy alone1 (1)
  Surgery and radiotherapy28 (29)
  Radiotherapy and chemotherapy3 (3)
  Surgery and chemotherapy1 (1)
  Radiotherapy, surgery, and chemotherapy1 (1)
Radiotherapy dose, mean, median (range), Gy49.6, 50 (30–70)
No treatment (n=3)
Follow-up, mean, median (range), ms (n=90)60, 45 (1.5–300)
  Recurrence or metastasis21 (23)
  No recurrence or metastasis69 (77)
  MM6 (7)
  AML1 (1)
Outcome (n=91)
  ANED63 (69)
  AWD13 (14)
  DOD6 (7)
  DOC9 (10)

[i] ms, months; MM, multiple myeloma; AML, acute myeloid leukaemia; ANED, alive, no evidence of disease; AWD, alive with disease; DOD, died of disease; DOC, died of other causes.

Case presentation

A 46-year old male presented to our hospital with cough and sore throat of a 4 month duration. He had a history of hypothyroidism for more than 10 years and received a diagnosis of tuberculosis before presenting to our hospital, but his symptoms persisted after anti-tuberculosis treatment. Fiberoptic laryngoscopy showed swelling of the epiglottis and aryepiglottic fold (Fig. 1). Laboratory findings showed an increased erythrocyte sedimentation rate, other examinations such as anti-tuberculosis antibody test and rheumatoid factors were normal. Chest X-ray was normal. Computed tomography (CT) and magnetic resonance imaging (MRI) of the neck revealed substantial swelling and edema of the epiglottis and enlargement of cervical lymph nodes. Biopsy of these two sites was performed under general anesthesia and microscopic observation showed many well-differentiated plasma cells and lymphocytes infiltration (Fig. 2). Immunohistochemical staining of the laryngeal specimen showed the most cells were positive for CD79a, CD138, CD38, CD5, Ki67, and Lambda, whereas negative for CD20, CD3, CD45RO, Cyclin D1, and PAX-5. Immunohistochemical staining of the cervical lymph nodes showed the most cells were positive for CD38, CD138, CD79a, CD45RO, CD31, Ki67, CD68 and LgG. Gene rearrangement studies indicated monoclonal rearrangements of the immunoglobulin heavy chain. A diagnosis of EMP of the larynx was made and a series of examinations were performed to exclude MM. Laboratory examinations including blood protein electrophoresis, serum immunoglobulins, urinary tests for Bence-Jones proteins were normal. Report of bone marrow biopsy was also within the normal range. In addition to cervical lymphadenopathy, PET-CT and other imaging examinations such as CT and MRI of the chest, abdomen and pelvis showed no distant metastasis. Complete surgical resection was not suitable for this patient, so, he was referred to the Hematology-Oncology Department, and received radiotherapy including 25 sessions of 55 Gy for laryngeal lesion and cervical metastasis. Meanwhile, adjuvant chemotherapy was also given with thalidomide, vincristine, epirubicin, and cyclophosphamide. His symptoms disappeared after treatment and he had monthly follow-ups.

Five years later, he was readmitted with dizziness that lasted 2 weeks. Complete blood count showed white blood cell 1.94×109/l, red blood cell 1.90×1012/l, haemoglobin 66 g/l, platelet 16×109/l. Bone marrow aspiration revealed a hyperplastic marrow: The granulocytes accounted for 29%, and the myeloblasts accounted for 12.5%; the mononucytes accounted for 32%, and the monoblasts and promonocytes accounted for 21% (Fig. 3). The blasts were positive for myeloperoxidase stain, and positive for nonspecific esterase, which was inhibited by sodium fluoride. Immunophenotyping of the bone marrow indicated that a group of blast cells (accounting for 4.84%) were positive for CD13, CD34, CD117, HLA-DR and negative for CD7, CD10, CD15, CD19, CD20, CD22, CD33, CD11b, CD14, CD64; another group of blast cells (accounting for 53.6%) were positive for CD13, CD33, CD15, CD64, CD11b, and weak positive for CD10 and CD14. These data are consistent with AML French-American-British (FAB) classification M4 subtype. Chromosome karyotype was 46, XY. Then CAG chemotherapy (aclarubicin hydrochloride, low-dose cytarabine and granulocyte colony-stimulating factor) combined with decitabine were administered accordingly and his condition alleviated. This patient is still being followed.

Patient demographics

We found greater higher occurrence in men and this was approximately two times more often than in women. The vocal cords and epiglottis are commonly involved and the main symptom is hoarseness often accompanied by dyspnea, dysphagia, and other symptoms. Supraglottic EMP accounted for the majority of patients with cervical lymphadenopathy. Likely this is due to association with lymphatic vascularity in the supraglottis, which is much denser than in the glottis or subglottis, and this causes greater incidence of lymph node metastasis.

Treatment options

Of 96 recorded treatment modalities, radiotherapy alone was the most common treatment modality, used in 41 cases, followed by a combination of surgery and radiotherapy, and surgery alone. Furthermore, we found that surgically based treatment was the most common treatment modality for cases published in recent years (Table III), despite there was no statistically significant difference between surgically based treatment and no-surgically based treatment modalities reported in these annual intervals (P=0.65).

Table III.

Treatment modalities by annual interval.

Table III.

Treatment modalities by annual interval.

Years

Treatment modality1948–19891990–19992000–20092010–2017
Surgically based treatment (%)22 (55)  7 (41)11 (46)9 (60)
No-surgically based treatment (%)18 (45)10 (59)13 (54)6 (40)
Outcomes and sequelaes

Overall treatment outcome was favorable, as a total of 84% of patients were alive after a mean follow-up of 60 months, independent of treatment modality. However, EMP outcomes for patients with cervical lymphadenopathy or multiple sites involvement were unfavorable, more than 40% with recurrence or metastasis during the limited follow-up period. A total of 21 patients were reported with relapse or metastasis in the clinical course, among which 12 cases were reported that EMP occurred in either multiple sites of the larynx or coexistence with other body sites, and 6 with cervical lymphadenopathy. A total of 6 cases developed MM finally, of which 3 cases occurred in the multiple sites of the larynx, and 2 originated in the supraglottis at the initial visits.

Discussion

EMP of the larynx is an extremely rare plasma cell neoplasm which constitutes less than 0.2% of the malignancies in the larynx (3,4). EMP may occur in various sites of the larynx such as the epiglottis, vocal folds, and subglottis. Clinical symptoms are closely related to the location of tumor and the degree of impairment of laryngeal structure. Laryngeal EMP may present different morphologic forms, sometimes a single, smooth polypoid mass, and sometimes diffuse swelling tissue just like our patient. So it is easily misdiagnosed due to the fact that the clinical symptoms and laryngoscope findings are nonspecific compared with other diseases such as laryngeal lymphoma and tuberculosis. Recently, imaging examination has been used more and more widely. For example, CT and MRI of neck may be used to identify the location of tumor and cervical lymphadenopathy, evaluate the involvement of the adjacent structures and curative effect. PET-CT has been used more and more to understand the nature of the lesion and the existence of the distant metastasis. Although radiological findings have acquired much achievement, diagnosis of EMP mainly relies on histopathologic examination by the presence of monoclonal plasma cell hyperplasia. However, the diagnosis could not be made early sometimes by routine pathological observation alone. Thus, immunohistochemistry and immunophenotype are proposed to make a definitive diagnosis or differential diagnosis, for example, most cells may be positive for CD138, CD38, CD79a, and negative for CD20, CD3 (3,4,35). Sometimes, immunoglobulin gene rearrangement analysis is also advised to confirm the diagnosis of EMP.

Given that EMPs are radiosensitive, radiotherapy is traditionally used as first-line treatment for solitary EMP (72). Similarly, single-modality radiotherapy was the most common treatment modality for laryngeal EMP, followed by a combination of surgery and radiotherapy, and surgery alone in our analysis. Recently, surgically based treatment, including surgical resection either alone or with adjuvant radiotherapy was proposed and proved that it could offer better survival outcomes compared to radiotherapy alone (3,73). In contrast, some studies showed no survival benefit for one treatment modality over another, and even recommended that radical surgery should be avoided for EMP (74). So far, the optimal treatment modality for the management of EMP remains controversial. But it has been generally accepted that chemotherapy is not considered to be a first-line therapy option and adjuvant chemotherapy is usually used in patients with disseminated or recurrent disease, that resembles the present case (3,72).

In our review, we found radiotherapy alone was the most common treatment modality for cases published between 1990 and 1999, but for cases reported from 2010 and onward, the most common treatment modality was surgically based treatment. There may be some reasons for the shift toward surgical management of small tumors. On the one hand, surgical techniques advance such as laser excision application for laryngeal microsurgery has made it possible to completely resection of lesion through minimally invasive surgery. On the other hand, patients receiving radiotherapy for head and neck EMP had a higher conversion to MM (3), and we found 4 of 6 patients that developed MM received radiotherapy alone in our review, therefore, surgical management of laryngeal EMP should be considered to avoid risk factors for conversion. However, whether it could offer better survival outcomes compared to radiotherapy alone is still to be further studied. Furthermore, patient outcomes may be associated with tumor distribution or cervical lymphadenopathy in addition to treatment modality. For example, more than 40% of patients with cervical lymphadenopathy or multiple sites involvement were reported with recurrence or metastasis, or even died of disease in our review. In summary, patient outcomes may be affected by many aspects, and management of laryngeal EMP should also be considered on a case-by-case basis. Factors such as tumor location; histological grade; regional lymphadenopathy; feasibility of complete resection; laryngeal function; and potential risk of recurrence or conversion to MM should be considered when determining the most suitable treatment modality.

EMPs tend to have more favorable outcomes than solitary bone plasmacytomas or MM, and overall survival for 10 years is estimated to exceed 70% (73). We noted that 84% of patients in our analysis were alive after a mean follow-up of 60 months. However, we also found that patients with cervical lymphadenopathy, multiple anatomical regions of the larynx or other organ involvement may be prone to relapse or metastasis. The highest risk of conversion to MM is reported to be in the first 2 years after diagnosis, but conversion has also been noted more than 15 years later (4). In our analysis, 3 patients developed MM in the first 2 years, and 1 subject developed MM 12 years later. Although there is debate about high risk factors of conversion to MM, once converted to MM, patients have poor prognosis, and fewer than 10% of patients survive 10 years (3). Therefore, progression to MM maybe a poor prognostic factor or a determinant factor for survival. Few patients developed MM in our analysis, and this was less than the expected range. This may be due to the relatively short follow-up for most cases. Therefore, follow-up and regular screening for MM is important.

To the best of our knowledge, this is the first case of laryngeal EMP who subsequently developed AML. On one hand, AML, as the primary second tumor, may occur subsequent to plasma cell myeloma or MM. On the other hand, the occurrence of AML in this case maybe closely related to chemotherapy or radiotherapy, and so it is referred to as therapy-related AML (t-AML). At this time, it is unclear whether this represents an intrinsic predisposition or therapy-related phenomenon (75). Similarly, the pathologic procedure and pathogenesis for this case are unclear and must be elucidated. Even so, this unusual case provides evidence that laryngeal EMP may develop therapy-related myeloid neoplasms (t-MNs) even though this is rare.

In conclusion, we present a comprehensive literature review spanning 60 years to increase awareness of laryngeal EMP. Our findings suggest radiotherapy alone is the most common treatment modality, but surgically based treatment has been the most common treatment modality in recent years. EMP localized to a single region of the larynx may have good outcomes. In addition to MM, t-MNs should be considered during the follow-up period. Due to the inherent limitations of this review, further study about optimal treatment modalities should be considered with randomized controlled clinical trials.

Acknowledgements

The authors would like to thank Dr Xunqiang Yin (School of Public Health, Central South University, Changsha, China) for his help in the statistical analysis of the paper and Professor Xinming Yang (Department of Otolaryngology-Head and Neck Surgery, The Second Xiangya Hospital, Changsha, China) for his assistance in the drafting and revision of the manuscript.

Funding

The present study was supported by the National Natural Science Foundation of China (grant nos. 81100360 and 30700940).

Availability of data and materials

All data generated or analyzed during the present study are included in this published article.

Authors' contributions

YY conceived this study, interpreted the results and revised the manuscript. SG analyzed the literature data and wrote the manuscript. GZ performed the data collection and analysis. All authors read and approved the final manuscript.

Ethics approval and consent to participate

This study was approved by the Ethics Committee of The Second Xiangya Hospital, Central South University and written informed consent was obtained from the patient.

Patient consent for publication

Written informed consent was obtained from the patient consent for the publication of their data and associated images.

Competing interests

The authors declare that they have no competing interests.

Glossary

Abbreviations

Abbreviations:

EMP

extramedullary plasmacytoma

MM

multiple myeloma

AML

acute myeloid leukemia

t-AML

therapy-related acute myeloid leukemia

MN

myeloid neoplasm

t-MNs

therapy-related myeloid neoplasms

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Spandidos Publications style
Ge S, Zhu G and Yi Y: Extramedullary plasmacytoma of the larynx: Literature review and report of a case who subsequently developed acute myeloid leukemia. Oncol Lett 16: 2995-3004, 2018
APA
Ge, S., Zhu, G., & Yi, Y. (2018). Extramedullary plasmacytoma of the larynx: Literature review and report of a case who subsequently developed acute myeloid leukemia. Oncology Letters, 16, 2995-3004. https://doi.org/10.3892/ol.2018.8992
MLA
Ge, S., Zhu, G., Yi, Y."Extramedullary plasmacytoma of the larynx: Literature review and report of a case who subsequently developed acute myeloid leukemia". Oncology Letters 16.3 (2018): 2995-3004.
Chicago
Ge, S., Zhu, G., Yi, Y."Extramedullary plasmacytoma of the larynx: Literature review and report of a case who subsequently developed acute myeloid leukemia". Oncology Letters 16, no. 3 (2018): 2995-3004. https://doi.org/10.3892/ol.2018.8992