Surgery is a Viable Treatment Option for Stage IE Primary Thyroid Lymphoma.

BACKGROUND: Stage IE primary thyroid lymphoma (PTL) has been diagnosed in approximately half of patients with PTL; however, the optimal treatment for stage IE PTL has not yet been established. METHODS: Stage IE PTL patients were identified from the Surveillance, Epidemiology, and End Results (SEER) database between 1998 and 2019. Thereafter, the disease-specific survival (DSS) and treatment modalities (surgery alone, surgery + radiotherapy (RT) and/or chemotherapy (CT), and RT and/or CT) of these patients were compared by Kaplan-Meier curves and log-rank test after propensity score matching (PSM). Additionally, patients with PTL from the Affiliated Sixth People's Hospital of the Shanghai Jiao Tong University and School of Medicine (Shanghai, China) between 2007 and 2022 were retrospectively analyzed as an external cohort. RESULTS: Among the 1596 patients with PTL from the SEER database, 842 were identified as patients with stage IE PTL, with an average follow-up period of 7.8 years. Pairwise analysis after PSM revealed no significant difference between the DSS of the three treatment groups. A total of 38 patients with PTL were identified in the external cohort, with an average follow-up period of 3.4 years. Compared with the RT and/or CT group, the surgery-alone group showed no significant difference in the incidence of hypothyroidism (p = 0.161) but had significantly fewer treatment-related complications (p = 0.021), shorter treatment duration (p < 0.001), and lower treatment costs (p = 0.025). CONCLUSIONS: The results of our study demonstrate that surgery is a viable treatment option for patients with stage IE PTL.

Primary Thyroid Lymphoma: A Retrospective-Observational Study in a Single Institutional Center.

Background and Objectives: primary thyroid lymphoma (PTL) is a rare neoplasm, displaying a variety of histological features. It is often a challenge for pathologists to diagnose this tumor. Materials and Methods: this study is a retrospective analysis of clinical and pathological characteristics of a group of eleven patients (eight women and three men, mean age 68 years, range 50-80 years) diagnosed with PTL. Results: nine patients (81.81%) presented a tumor with progressive growth in the anterior cervical region, usually painless and accompanied by local compressive signs. Histologically, we identified six cases (55%) of diffuse large B-cell lymphoma, three cases (27%) of extranodal marginal zone lymphoma, one case (9%) of follicular lymphoma, and one case (9%) of mixed follicular-diffuse lymphoma. PTL was associated with microscopic Hashimoto autoimmune thyroiditis in ten cases (90.9%). Ten patients (90.9%) presented with localized disease (stage I-IIE). A percentage of 60% of patients survived over 5 years. We observed an overall longer survival in patients under 70 years of age. Conclusions: PTL represents a diagnosis that needs to be taken into account, especially in women with a history of Hashimoto autoimmune thyroiditis, presenting a cervical tumor with progressive growth. PTL is a lymphoid neoplasia with favorable outcome, with relatively long survival if it is diagnosed at younger ages.

Diagnosis and treatment of primary thyroid lymphoma from a surgical perspective: a multi-institutional study.

PURPOSE: Surgery of primary thyroid lymphoma (PTL) has been mostly limited to diagnostic work-up. This study aimed to further study its potential role. METHODS: This was a retrospective study from a multi-institutional registry of PTL patients. Clinical, diagnostic work-up (fine needle aspiration, FNA; core needle biopsy, CoreNB), contribution of surgery (open surgical biopsy, OpenSB; thyroidectomy), histology subtype, and outcome data were evaluated. RESULTS: Some 54 patients were studied. Diagnostic work-up included FNA in 47 patients, CoreNB in 11, and OpenSB in 21. CoreNB yielded the best sensitivity (90.9%). Thyroidectomy was performed in 14 patients with other diagnosis (incidental PTL), in 4 for diagnosis and in 4 for elective treatment of PTL. Incidental PTL was associated with not performed FNA nor CoreNB (OR 52.5; P = 0.008), mucosa-associated lymphoid tissue (MALT) subtype (OR 24.3; P = 0.012), and Hashimoto's thyroiditis (OR 11.1; P = 0.032). Lymphoma-related death (10 cases) mostly occurred within the first year after diagnosis and was associated with diffuse large B-cell (DLBC) subtype (OR 10.3; P = 0.018) and older patients (OR 1.08 for every 1-year increase; P = 0.010). There was a trend towards lower mortality rate in patients receiving thyroidectomy (2/22 versus 8/32, P = 0.172). CONCLUSION: Incidental PTL accounts for most of thyroid surgery cases and are associated with incomplete diagnostic work-up, Hashimoto's thyroiditis and MALT subtype. CoreNB appears to be the best tool for diagnosis. Most of PTL deaths occurred during the first year after diagnosis and mostly related to systemic treatment. Age and DLBC subtype are poor prognostic factors.

Differential diagnosis of non-diffuse primary thyroid lymphoma and papillary thyroid carcinoma by ultrasound combined with computed tomography.

BACKGROUND: Primary thyroid lymphoma (PTL) and papillary thyroid carcinoma (PTC) are both thyroid malignancies, but their therapeutic methods and prognosis are different. This study aims to explore their sonographic and computed tomography(CT)features, and to improve the early diagnosis rate. METHODS: The clinical and imaging data of 50 patients with non-diffuse PTL and 100 patients with PTC confirmed by pathology were retrospectively analysed. RESULTS: Of the 150 patients, from the perspective of clinical data, between non-diffuse PTL and PTC patients existed significant difference in age, maximum diameter of nodule, asymmetric enlargement and Hashimoto's thyroiditis (P < 0.001), but not in gender ratio, echo texture, cystic change and anteroposterior-to-transverse ratio (P > 0.05). With respect to sonographic feature, non-diffuse PTL patients had a higher proportion than PTC patients in markedly hypoechoic, internal linear echogenic strands, posterior echo enhancement, rich vascularity, lack of calcification and homogeneous enhancement, with statistically significant difference (P < 0.05), while PTC patients had a higher proportion than non-diffuse PTL patients in irregular border, circumscribed margin, capsular invasion and significant enhancement, with statistically significant difference (P < 0.001). With respect to CT feature, non-diffuse PTL patients were significantly different from PTC patients in the non-contrast CT value mean, venous phase CT value mean, enhanced intensity and homogeneity of nodules (P < 0.05). Multivariate logistic regression analysis showed that age (OR = 1.226, 95%CI:1.056 ~ 1.423, P = 0.007), posterior echo enhancement (OR = 51.152, 95%CI: 2.934 ~ 891.738, P = 0.007), lack of calcification (OR = 0.013, 95%CI: 0.000 ~ 0.400, P = 0.013) and homogeneous enhancement (OR = 0.020, 95%CI: 0.001 ~ 0.507, P = 0.018) were independent risk factors. CONCLUSIONS: Sonographic and CT features of the presence of posterior echo enhancement, lack of calcification and homogeneous enhancement were valuable to distinguishing non-diffuse PTL from PTC.

Prognostic evaluation models for primary thyroid lymphoma, based on the SEER database and an external validation cohort.

PURPOSE: Primary thyroid lymphoma (PTL) is a rare malignancy, and the literature is limited to small case series and case reports. This study aimed to assess the epidemiologic characteristics, survival, and prognostic factors of patients with PTL. METHODS: We analyzed 2215 PTL patients from the Surveillance, Epidemiology, and End Results database medical records, between 1983 and 2015, as the training cohort. We enrolled 105 patients from the Cancer Hospital, Chinese Academy of Medical Sciences, for the external validation cohort. The nomograms for predicting the 1-, 5-, and 10-year overall survival (OS) and lymphoma-specific survival (LSS) were constructed. RESULTS: PTL incidence steadily increased from 1977 to 1994, with an annual percentage change of 3.2% (95% confidence interval [CI]: 1.2-5.2, P < 0.05). The 1-, 5-, and 10-year OS and LSS rates were 84.66%, 71.61%, and 55.95%; and 90.5%, 85.7%, and 82.2%, respectively. Multivariate Cox regression analysis revealed that shorter OS association with age ≥ 60 years (hazard ratio [HR], 3.94; 95% CI 3.31-4.69; P < 0.001), unmarried status (HR, 1.55; 95% CI 1.37-1.75; P < 0.001), Ann Arbor stage III-IV (HR, 1.55; 95% CI 1.37-1.75; P = 0.020), diffuse large B-cell lymphoma (HR, 2.60; 95% CI 1.15-5.87; P = 0.022), and T cell non-Hodgkin lymphoma (HR, 3.53; 95% CI 1.12-11.10; P = 0.031). In the multivariate competing-risk analyzes, age, stages III-IV, year of diagnosis, surgery, radiation, chemotherapy, and histology were strongly predictive of PTL-specific risk of death. To estimate the 1-, 5-, and 10-year LSS and OS rates, respectively, nomograms were built. In the validation cohort, the results also confirmed the utility. CONCLUSIONS: This study presents the first prognostic model with an external validation that could help clinicians identify patients with high-risk PTL to improve their prognosis.

Multimodal Sonographic Appearance and Survival Outcomes of 69 Cases of Primary Thyroid Lymphoma Over 10 Years.

OBJECTIVES: To investigate ultrasound appearance and the survival outcomes for patients with primary thyroid lymphoma (PTL). METHODS: Ultrasonic images and clinical characteristics from pathologically confirmed 69 PTL patients (2008-2019) were retrospectively analyzed. The clinical characteristics, ultrasonic characters, and prognostic factors were analyzed. Survival curves were plotted using the Kaplan-Meier method. Univariate and multivariate analyses were performed. RESULTS: Of the 69 study patients, 23 were indolent PTL and 46 were aggressive PTL. Age (>70 years old) and elevated lactate dehydrogenase levels were statistically different clinical features between aggressive and indolent PTL. From ultrasonic images, 34 cases were nodular, 11 diffuse, and 24 mixed pattern. Mixed types displayed high invasiveness (45.7%) while diffuse types displayed higher inertness (39.1%), with statistically significant differences (P = .000). Invaded thyroid capsule and increased chaotic vascularity also showed significant differences between aggressive and indolent PTL. We also observed statistical difference in overall survival rates between aggressive and indolent PTL (P = .032). Single factor K-M analyses showed that age >70 years, aggressive pathology, and Ki67 >30% were positively correlated with the risk of poor PTL survival (P < .05). CONCLUSIONS: Multimodal ultrasound provides accurate ultrasonographic information and facilitates PTL invasiveness diagnostics for improved clinical treatment. In addition, PTL patients aged >70 years, with aggressive pathology, and Ki67 >30% were more likely to have a poor survival outcome.

Primary thyroid lymphoma: Case series of patients from a developing country.

Primary Thyroid Lymphoma (PTL) is a rare disease, mostly affecting middle-to-old aged females. It has a strong association with pre-existing thyroiditis, which increases the risk of its development. The disease has been studied in detail in the Western populations. However, there is lack of data for south Asians, especially in Pakistani population. This study was carried out to learn more about the disease characteristics in our area. This was a retrospective study where we reviewed four diagnosed and/or treated cases of primary thyroid Lymphoma at tertiary care hospital of Pakistan (Aga Khan University Hospital), Karachi, Pakistan. Each patient's medical record was reviewed and studied. The participants' average age was 62.8±10.12 years,, with two males and two females. The histology of all of the patients was compatible with the Diffuse B cell Lymphoma subtype. One patient died as a result of complications, one patient was lost to follow up, and the remaining two patients are doing well with no active complaints and are being followed up on a regular basis.

Lymphomas and thyroid: Bridging the gap.

The thyroid gland is often involved in the development of neoplastic diseases, including lymphoproliferative disorders. The aim of this paper is to provide a comprehensive overview of the multiple interactions between lymphoma and thyroid. Through an extensive research among the literature, the relationship between lymphomas and thyroid can be established at various levels, and the possible interconnections are here summarized in 5 points: (1) the greater risk of lymphoma development in some thyroid diseases; (2) the primary thyroid lymphoma, with focus on issues related to the diagnosis, differential diagnosis, and treatment; (3) the incidence of thyroid involvement in primitive nodal lymphomas or in extranodal lymphomas of the other sites; (4) thyroid changes after treatment of lymphoma, in relation to the effects of radiation therapy and immuno-chemotherapy; (5) the incidental findings of thyroid changes, on imaging, in patients with lymphoma, without a direct involvement of the gland in malignant disease. In conclusion, issues that until now have been dealt with separately will therefore be analyzed in a unique paper, allowing a global view of the topic and emphasizing the need of a multidisciplinary approach. Future learning areas in this topic mainly relate to rapidly increasing the knowledge of imaging studies together with expanding the armamentarium of novel biological and targeting agents in lymphoma patients.

Eleven patients with primary thyroid lymphoma: a single center experience

Background/aim: Primary thyroid lymphoma (PTL) is a very rare thyroid malignancy. It should be diagnosed and treated immediately and accurately. Our aim was to evaluate the diagnostic methods and treatment results for patients with PTL.Materials and methods: We retrospectively evaluated the records of 11 patients with PTL from 2009 to 2015, diagnosed at our institute. Age, sex, stage, histopathologic type, presence of Hashimoto's thyroiditis, diagnostic methods, treatment types, and recurrence were examined.Results: Six patients were female, 5 were male, and the median age of the patients was 61 years (range: 15-76 years). All patients had a large palpable mass in the neck. Fine needle aspirate (FNA) biopsy was performed in all patients; however, it was useful only in the diagnosis of 7 patients. Excisional and surgical biopsy was performed in 4 patients. All patients had non-Hodgkin B-cell lymphoma, including 9 cases of diffuse large B-cell lymphoma (DLBCL), and 2 patients had mucosa-associated lymphoid tissue (MALT) lymphoma. Recurrence was observed in one patient. Median survival was 34 months.Conclusions: The preferred option for the diagnosis of PTL should be FNA biopsy, and the treatment should be decided on according to whether the disease is limited to the thyroid gland or not, its histological type, and its stage.

Wnt5a and Ror2 expression associate with the disease progress of primary thyroid lymphoma.

Primary thyroid lymphoma (PTL) is a rare malignant thyroid tumor; its pathogenesis is closely related to chronic lymphocytic thyroiditis. The different pathological subtypes and stages of PTL have distinct clinical characteristics and prognosis, but the specific reasons are not clear. Wnt5a is a representative protein of non-canonical Wnt signaling. It plays an important role in many different types of tumors. This study is to explore the changes of Wnt5a and its receptor Ror2 in PTL development process and the clinical significance of their represent. We collected 22 PTL patient tumor specimens and clinical data. We observed the expression of Wnt5a and Ror2 in PTL tumor tissues by immunohistochemistry. Wnt5a was expressed positively in 12 (54.5 %) cases, and Ror2 was expressed positively in 18 (81.8 %) cases. The expression of Wnt5a had a significant difference in different pathological subtypes of PTL (P < 0.05). Wnt5a and Ror2 expression were associated with local invasion and clinical stage, respectively (P < 0.05), and had no significant correlation with age, gender, and tumor size. Although, no significant difference in overall survival was found between positive and negative groups of Wnt5a (P = 0.416) or Ror2 (P = 0.256), respectively. We still consider that Wnt5a and Ror2 play a complex and subtle role in the pathogenesis and progression of PTL and may become potential biomarkers and therapeutic targets of PTL.

12 cases of primary thyroid lymphoma in China.

INTRODUCTION: Primary thyroid lymphoma (PTL) is a rare malignant disease, which can be life threatening because of airway obstruction due to a suddenly and rapidly growing mass. Prompt and accurate detection and diagnosis in the early phase of PTL are crucial for the treatment of this disease. However, due to lack of standardized diagnostic procedures and methods, PTL can be easily missed or misdiagnosed. MATERIALS AND METHODS: In this study, we retrospectively reviewed 12 thyroid lymphomas cases to determine the typical clinical and sonographic profiles of thyroid lymphomas. RESULTS: All the patients showed symptoms of rapidly developing neck swelling or mass sensation when they underwent diagnostic procedures. We found that, in sonography, 3 of the 12 patients (25.0 %) showed diffuse heterogeneous hypoechoic parenchyma with intervening echogenic septa-like structures, 8 (66.7 %) showed markedly hypoechoic masses, and 1 (8.3 %) showed a mixed pattern. Histological analysis of the lymphomas using paraffin-embedded sections revealed that all the 12 lymphomas were non-Hodgkin lymphoma with a B cell origin: extranodal marginal zone B cell lymphomas of the mucosa-associated lymphoid tissue lymphoma type in 3 (MALT, 25.0 %), and diffuse large B cell lymphomas in 9 (75.0 %) lymphomas. All the MALT patients had diffuse heterogeneous hypoechoic parenchyma in sonography, but only one of them with diffuse large B cell lymphomas had diffuse heterogeneous hypoechoic parenchyma in sonography. Complete remission was achieved in all patients when they were given CHOP or COP therapy accompanied by local radiotherapy after accurate diagnosis of PTL. CONCLUSION: We analyzed clinical, sonographic and histological features of twelve cases of malignant lymphoma arising in the thyroid gland and proposed a systematic strategy for prompt and accurate diagnosis of PTL.

The potential value of ultrasound in predicting local refractory/relapse events in primary thyroid lymphoma patients.

BACKGROUND: Primary thyroid lymphoma (PTL) is a rare malignant disorder, and ultrasound plays an important role in PTL diagnosis and follow-up surveillance. Prediction of refractory/relapse events in PTL patients is an essential issue, yet no ultrasonic PTL features have been discovered to be related to refractory/local relapse events. METHODS: From January 2008 to September 2022, newly diagnosed PTL patients in our center who underwent standard first-line treatment and received an ultrasound examination before treatment were enrolled. Data regarding patients' clinical and sonographic features, as well as their therapeutic responses were collected. Subjects with an ideal prognosis were compared to those with refractory/relapse events. RESULTS: In total, 37 PTL patients were analyzed, including 26 with diffuse large B-cell lymphoma, 2 with follicular lymphoma and 9 with mucosa-associated lymphoid tissue lymphoma. During the median follow-up of 25 months, 30 patients obtained a complete response, 4 were refractory patients, and 3 experienced local relapse. No significant difference was detected in the baseline clinical characteristics between patients with an ideal prognosis and those with refractory/local relapse events. In terms of sonographic features, however, an event-free survival (EFS) curve comparison revealed that patients with bilobar enlargement (defined as an anterior-posterior diameter > 2.5 cm on both sides of thyroid lobes) had a poorer EFS than those without (P < 0.0001), and patients with diffuse type had a poorer EFS than those with mixed/nodular types (P = 0.043). No significant difference was observed in EFS between patients with or without signs of suspicious cervical lymph node metastasis, rich blood signal distribution or symptoms of trachea compression. CONCLUSIONS: PTL patients with an anterior-posterior diameter > 2.5 cm for both thyroid lobes or PTL patients of the diffuse ultrasound type could be prone to refractory/local relapse events.

Rare Thyroid Diseases Mimicking or Concealing Primitive Thyroid Neoplasms: A Call for a "Check and Double-Check" Clinical Mindset.

Clinical endocrinologists encounter in their practice patients with thyroid diseases on a daily basis. Still, diagnosis of rare structural thyroid disorders can be quite challenging. In some instances, they do not only impersonate but can also conceal, other conditions such as thyroid carcinomas. We describe a series of patients with structural thyroid disorders including 1) anaplastic thyroid carcinoma initially presenting with features of thyroid abscess; 2) unicentric hyaline vascular Castleman's disease of the thyroid embedded in a stroma of papillary thyroid carcinoma; and 3) primary thyroid lymphoma with a rapid and fulminant evolution. The common challenge in the diagnosis of these cases lies in both their low incidence and their complex presentation. We use the presentation of these cases to raise the attention related to their identification. We highlight the need for precision diagnosis to enable a patient-tailored management approach and improve patient outcomes.

[Clinical characteristics and prognostic analysis of 34 patients with primary thyroid lymphoma].

Objective: To investigate the clinical characteristics, diagnosis, treatment, and prognosis of primary thyroid lymphoma (PTL) . Methods: A retrospective analysis was conducted on the clinical and pathological data of 34 newly diagnosed PTL patients admitted to Beijing Tongren Hospital from September 2010 to February 2023. The Kaplan-Meier survival curve and Log-rank test were used for survival analysis, and the Cox regression model was applied for univariate analysis of prognostic factors. Results: All 34 PTL patients presented with cervical mass as the initial clinical manifestation. There were 9 males and 25 females. The pathological diagnosis was diffuse large B-cell lymphoma (DLBCL) in 29 patients and mucosa-associated lymphoid tissue (MALT) lymphoma in 5 patients. Among the DLBCL patients, 6 had B symptoms, 17 had an Eastern Cooperative Oncology Group (ECOG) score of ≥2, the Ann Arbor staging was stage Ⅰ-Ⅱ in 21 cases and stage Ⅲ-Ⅳ in 8 cases, the tumor diameter was ≥10 cm in 4 cases, and 14 had concurrent Hashimoto thyroiditis; 27 cases received chemotherapy, with 21 cases achieving complete remission (CR), 2 cases partial remission (PR), and 6 cases of disease progression; the 5-year progression-free survival and overall survival rates were 78.9% and 77.4%, respectively; univariate survival analysis showed that B symptoms, tumor diameter ≥10 cm, and Ann Arbor stage Ⅲ-Ⅳ were significant factors affecting patient prognosis (P<0.05). MALT lymphoma patients were all in stages Ⅰ-Ⅱ, had an ECOG score of 0-1, and were without B symptoms. All patients underwent surgical resection, with 4 cases achieving CR and 1 case PR. Conclusion: PTL is more common in females with concurrent Hashimoto thyroiditis, with the majority of pathological types being B-cell lymphoma. The main treatment is chemotherapy, supplemented by radiotherapy and surgery, and the prognosis is relatively favorable.

Thyroid B-Cell Lymphoma in the Background of Hashimoto's Thyroiditis: A Case Report and Literature Review.

Primary thyroid lymphoma (PTL) is a rare type of thyroid cancer, comprising less than 5% of all thyroid cancer cases. PTL includes subtypes like diffuse large B-cell lymphoma (DLBCL) and mucosa-associated lymphoid tissue lymphoma (MALT). The connection between PTL and autoimmune diseases of the thyroid, particularly Hashimoto's thyroiditis, has gained recognition in recent years. Studies have indicated an increased incidence of PTL among individuals with Hashimoto's thyroiditis. However, effectively recognizing and managing PTL in the context of autoimmune thyroid diseases remains challenging. Further research and clinical experience are needed to develop comprehensive strategies for early detection and optimal management of this complex condition. In a case involving an 88-year-old female diagnosed with diffuse large B-cell lymphoma, she presented with a complaint of persistent neck swelling for five years. The patient also experienced symptoms such as dysphagia, hoarseness of voice, obstructive sleep apnea, and choking attacks. Surgical resection of the neck swelling was successfully performed, and the patient was referred to the oncology department for further treatment. Thyroid B-cell lymphoma is an exceedingly rare form of thyroid cancer, typically identified in individuals who have a history of Hashimoto's thyroiditis. The prognosis for thyroid B-cell lymphoma is generally unfavorable, and surgical intervention remains the primary treatment approach for such cases.

Two lymphoma histotypes and papillary thyroid carcinoma coexisting on Hashimoto ground: a case report and review of the literature.

BACKGROUND: Papillary carcinoma is the most frequent type of thyroid carcinoma, while primary thyroid lymphoma is uncommon disease. The coexistence of these entities has already been described, and the common risk factor is considered Hashimoto thyroiditis. The two most frequent histotypes of primary thyroid lymphoma are diffuse large B-cell and mucosa-associated lymphoid tissue lymphoma, but the coexistence of both with papillary carcinoma is rarely reported. METHODS: We present a case of a previously healthy 57-years old male with rapidly growing lump on the right side of the neck. Ultrasonography revealed nodules in both thyroid lobes. Fine needle aspiration cytology and pertechnetate scintigraphy were performed. Due to the Bethesda T-5 in the "cold" nodule of the right lobe, surgery with histopathological and immunohistochemistry analysis was indicated. RESULTS: Histopathological and immunohistochemistry methods confirmed concomitant malignancies in the thyroid gland: diffuse large B-cell lymphoma and papillary carcinoma in the right, and mucosa-associated lymphoid tissue lymphoma in the left lobe with Hashimoto thyroiditis in the remaining tissue. Patient underwent therapy procedures and was without signs of local recurrence or metastatic spread on subsequent follow-up. CONCLUSIONS: Sudden appearance of the neck mass in patients with Hashimoto thyroiditis should raise suspicion on primary thyroid lymphoma and be promptly taken in the diagnostic workup, including fine needle aspiration cytology. Pathology with immunohistochemistry is crucial for further clinical decision making. Since the standardized protocol in management of these complex patients is missing, personal approach and close collaboration between cytologist, pathologist, surgeon, haematologist and nuclear medicine specialist is essential.

Thyroid lymphoma: recent advances in diagnosis and optimal management strategies.

Primary thyroid lymphoma is rare, composing approximately 5% of all thyroid malignancies and less than 3% of all extranodal lymphomas. It typically presents as a rapidly enlarging goiter with associated compressive symptoms. Thyroid ultrasound and fine needle aspiration cytology, using flow cytometry and immunohistochemistry, remain the main modalities used to confirm the presence of lymphoma. The increasing use of an ultrasound-guided core biopsy to achieve an accurate diagnosis has further limited the role of surgery. An open surgical biopsy may still be required not only for definitive diagnosis but also to confirm the subtype of lymphoma. There are limited numbers of randomized or prospective trials to guide management, and controversy remains over optimal treatment. Treatment and prognosis of this disease can be dichotomized into two separate groups: pure mucosa-associated lymphoid tissue (MALT) lymphoma and diffuse large B-cell lymphoma (DLBCL) or mixed subtypes. Early stage (stage IE) intrathyroidal MALT lymphomas typically have an indolent course and may be treated with single-modality surgery, radiotherapy, or a combination of both. DLBCLs are more aggressive, and survival outcomes are highest with multimodal therapy incorporating monoclonal antibodies, chemotherapy, and radiotherapy. The prognosis is generally excellent but can be varied because of the heterogeneous nature of thyroid lymphomas. The aim of this paper is to discuss the changes in diagnostic modalities and to focus on the recent alterations in the management of this rare disease, including targeted therapies as well as the more limited role of the endocrine surgeon.

Rapidly Growing Goitre: Not Always A Carcinoma.

Primary thyroid lymphoma (PTL) is a rare malignancy of the thyroid gland which should be considered in patients with a rapidly growing cervical mass, especially in those with a history of Hashimoto's thyroiditis. We present the case of a 53-year-old woman with a rapidly growing goitre with compressive symptoms. Computed tomography (CT) imaging was performed to examine the extent of disease and a biopsy showed B-cell non-Hodgkin lymphoma, stage I according to the Ann Arbor classification. The patient was immediately treated with high-dose prednisone for her compressive symptoms and after the diagnosis she received six cycles of R-CHOP (rituximab-cyclophosphamide-doxorubicin-vincristine-prednisone) chemotherapy. The patient has now been in remission for 12 months. We present this case to demonstrate the importance of awareness of PTL. Up to 10% of cases can be missed with fine needle aspiration cytology (FNAC), making it important to obtain a histological biopsy in rapidly growing goitres. In addition, establishing the correct diagnosis can in most cases prevent unnecessary surgical intervention. The preferred treatment with the best overall survival is chemotherapy with or without radiation therapy. LEARNING POINTS: Primary thyroid lymphoma is a rare malignancy of the thyroid gland which should be considered in rapidly growing goitres, especially when there is a history of Hashimoto's thyroiditis.A histological biopsy is preferred to minimize misdiagnoses.Surgical intervention can typically be avoided with correct diagnosis and with the use of corticosteroids to reduce compression symptoms.

Intra-Abdominal Paraganglioma and Primary Thyroid Lymphoma in a Single Patient: The First Case Report.

Rare diseases such as primary thyroid lymphoma (PTL) and paragangliomas exist. Although only 0.5% of patients experience a transformation from thyroiditis to PTL, patients with Hashimoto's thyroiditis have a higher risk of developing PTL than the general population. Primary non-Hodgkin lymphoma of the thyroid is rare. Paragangliomas are neuroendocrine tumors that originate from chromaffin cells situated along the sympathetic and parasympathetic chains. This paper reports the first case of primary diffuse large B-cell lymphoma with nonfunctional paraganglioma. A 29-year-old female presented with an enlarged neck and recurrent compressive symptoms. Ultrasonography results showed a nodule in the right lobe of the thyroid gland. Emergency thyroidectomy was performed after obtaining inconclusive fine-needle aspiration results. Immunohistopathology of the biopsy specimen confirmed the presence of a large diffuse B-cell lymphoma. Computed tomography revealed a nonfunctional mass in the adrenal gland. The team then proceeded with the appropriate treatment.

Primary Thyroid Diffuse Large B-cell Lymphoma in a Child with Hashimoto's Thyroiditis: A Case Report

Primary thyroid lymphoma (PTL) is a rare thyroid gland cancer, with diffuse large B-cell lymphomas (DLBCL) being extremely rare in children and adolescents. Thus, optimal therapy is debatable. We describe a rare case of thyroid DLBCL in an adolescent girl with a history of Hashimoto thyroiditis (HT), the difficulty in diagnosis and the outcome of treatment. A 12-year-old girl with a nine-year history of HT was admitted with a right-sided painless progressive swelling of the neck. Physical examination and imaging including ultrasound (US), computed tomography (CT) and positron emission tomography/CT revealed an enlarged thyroid gland with right side lymphadenopathy and no metastasis. Two fine needle aspirations were done showing suspected lymphoblastic lesions for non-Hodgkin lymphoma without precise diagnosis. US guided core needle biopsy was finally performed confirming the diagnosis of DLBCL. She was treated according to LMB 96-group B protocol with no surgical removal of thyroid. The patient responded very well to treatment and 14 months later there is no evidence of relapse or metastases. PTL is an extremely rare cause of thyroid malignancy in children. However, it should be considered in the differential diagnosis of a thyroid mass in adolescents presenting with a rapidly enlarging neck mass and a history of HT. It is a treatable condition with a good prognosis, even in aggressive histological subtypes, with no need for thyroidectomy.