Primary Non-Hodgkin Research Articles

Primary Gastrointestinal Non-Hodgkin’s Lymphoma of the Small and Large Intestines: a Systematic Review

BackgroundPrimary gastrointestinal non-Hodgkin’s lymphoma (PGINHL) of small and large intestines is a group of heterogeneous, rare malignancies. Optimal treatment practices remain undefined. MethodsA systematic review (2003–2015) was performed to assess tumor characteristics, treatment practices, and treatment outcomes of PGINHL of small and large intestines. ResultsTwenty-eight studies (1658 patients) were included; five focused on follicular lymphoma subtype. Of the non-follicular patients, 59.3 % presented with abdominal pain, 37.2 % were located in ileocecum, and 53.6 % were diffuse large B cell lymphoma subtype. The majority of patients (60.7 %) were treated with a combination of surgery and chemotherapy. Forty-three percent of studies concluded an overall survival benefit with surgery; none reported increased postoperative morbidity or mortality. Survival outcomes were not typically stratified by emergent versus elective surgery. Multivariate analysis within individual studies associated B cell lymphoma and ileocecum location with higher survival, while advanced stage and B symptoms were associated with poorer survival. Patients with asymptomatic follicular lymphoma had no progression with a watchful waiting approach. ConclusionsThe majority of patients with non-follicular small and large intestinal PGINHLs are treated with both chemotherapy and surgery. Although surgery appears to be an important part of the treatment algorithm, definitive statements regarding its survival benefit remain limited due to lack of patient stratification based on timing and indication for surgery.

Primary Non-Hodgkin Lymphoma of the Breast

Lymphomas constitute approximately 0.15% of malignant mammary neoplasms. Less than 0.5% of all malignant lymphomas involve the breast primarily. Primary non-Hodgkin breast lymphoma is usually right sided. The combined therapy approach, with chemotherapy and radiotherapy, is the most successful treatment. Mastectomy offers no benefit in the treatment of primary non-Hodgkin breast lymphoma. To the author's knowledge, this is the first published case of primary non-Hodgkin breast lymphoma reported with conventional ultrasonography, elastography (both freehand and acoustic radiation force impulse imaging), digital mammography, contrast-enhanced digital mammography, and pathology findings. A 45-year-old woman presented with a lump in the right breast for 2 months. There was no evidence of systemic lymphoma or leukemia when the breast lesion was detected. Imaging findings were negative for lymphoma. Ipsilateral lymph nodes were not palpable. The mass was resected, and histopathology findings were diagnostic of non-Hodgkin lymphoma. Immunohistochemistry was confirmatory of non-Hodgkin lymphoma, diffuse large cell type of B-cell lineage. Although primary and secondary lymphomas of the breast are rare entities, they should be considered in the differential diagnosis of breast malignancies.

Primary Liver Sinusoidal Non-Hodgkin's Lymphoma Presenting as Acute Liver Failure

We describe a case of a middle-aged woman, who presented to us with fever, anorexia, abdominal distension from a massive hepatomegaly, low hemoglobin, and acute liver failure. A liver biopsy revealed B cell non-Hodgkin's lymphoma predominantly in the sinusoids with CD10, CD20, and Bcl-2 positive on immunohistochemistry. She initially responded well to chemotherapy but succumbed 6 months later to the recurrence of disease. Sinusoidal non-Hodgkin's lymphoma of the liver should be considered in the differential diagnosis of a patient with large hepatomegaly presenting with acute liver failure.

Primary Non-Hodgkins lymphoma of the parotid gland

AbstractPrimary malignant lymphomas in the salivary glands are relatively rare. Clinical presentation is not characteristic and the disease is often overlooked resulting in diagnosis and treatment delays. AimTo stress the importance of the diagnostic process and combined management, we present a series of eight patients with malignant lymphoma of the parotid who were diagnosed only after surgery and managed with radiation and chemotherapy. MethodsRetrospective series of patients with primary malignant lymphoma of the parotid gland managed with radiotherapy and diagnostic surgical partial resection. ResultsAfter treatment completion we achieved a loco-regional control rate of 87.5%. Toxicity was charted according to the Common Toxicity Criteria and it was seen in six patients (75%). Six patients are still alive without evidence of recurrent disease in their last follow-up. ConclusionOur study confirms that primary early stage Non-Hodgkin Lymphoma of the parotid gland is a disease with an excellent prognosis and a good local control rate, with minimal morbidity.

The Role of Surgery in the Clinical Management of Primary Gastrointestinal Non-Hodgkin's Lymphoma

Primary gastrointestinal non-Hodgkin's lymphoma (PGINHL) is a heterogeneous family of tumors, with treatment modalities including chemotherapy, surgery, and radiotherapy. Because the role of surgery in PGINHL remains disputed, this study aims to assess the impact of operative resection on survival. We used a pathology database to identify all cases of PGINHL diagnosed at a single academic-affiliated medical center from 1988 to 2013. Demographic and clinical data were abstracted from the medical record. We summarized the clinical courses of patients with PGINHL and then performed a survival analysis to compare overall and disease-free survival, stratified by demographic and clinical variables. We identified 33 patients diagnosed with PGINHL during the study period. Of 29 who subsequently received treatment at the institution, 15 initially underwent chemotherapy, 10 underwent surgical resection, and 4 underwent surgery for other reasons such as diagnosis without resection or management of disease complications. Three patients suffered surgical complications and two of these patients died. We found no difference in overall survival between patients receiving surgical resection and patients managed initially with chemotherapy. This case series supports a continued role for surgical resection in the management of patients with PGINHL, though anticipated benefits should be weighed against the risk of complications.

Primary Thyroid Non-Hodgkin’s Lymphoma

Primary non-Hodgkin’s lymphoma of the thyroid gland was rarely described. We report the case of a 44-year-old man admitted for an anterior neck swelling, hoarseness and dyspnea. The chest radiograph showed a trachea deviation. He had no clinical, biological or radiological sign of other lymphoma locations. Ultrasound examination of the neck revealed a bilateral heterogeneous thyroid lesion. Cytology revealed lymphoid cells having high nuclear-cytoplasmic ratio with multiple and irregular nucleoli. An urgent thyroid surgery consisting of total thyroidectomy had been performed since the presence of compressive signs due to the tumor. The histopathological examination of a biopsy from the thyroid tissue confirmed a high-grade non-Hodgkin’s lymphoma. Then, L-thyroxin substitution therapy, chemotherapy and radiotherapy were initiated. A prolonged remission was noted.

Primary Bilateral Non-Hodgkin's Lymphoma of the Adrenal Gland Presenting as Incidental Adrenal Masses.

Although lymphoma may occasionally involve the adrenal glands as part of a generalized disease process, primary adrenal lymphoma (PAL) is a rare disease. We present a case of a 62-year-old woman with a history of mild/moderate hereditary spherocytosis with a well-compensated baseline haemoglobin, who presented with rapidly progressive symptomatic anaemia. During the diagnostic workup, imaging revealed bilateral large adrenal masses and she was later diagnosed with diffuse large B-cell non-Hodgkin's lymphoma (DLBCL), with the adrenal glands being the dominant site of the disease. The patient was started on systemic chemotherapy, but her disease progressed with neurological involvement which responded to second-line therapy. Her adrenal disease however was refractory to further therapy.

Primary T-Cell Non-Hodgkin Lymphoma of the Vagina.

The primary vaginal T-cell non-Hodgkin lymphoma is a rare form of lymphoma. Most of the previously published cases were about B-cell non-Hodgkin lymphomas. We present the case of a vaginal mass in an 82-year-old patient presenting vaginal bleeding. The results of the immunohistological studies of the mass revealed the presence of a cytotoxic T-cell non-Hodgkin lymphoma, which is the least common subtype.

Primary B-cell Non-Hodgkin Lymphoma of Gallbladder Presenting as Cholecystitis.

Primary B-cell Non-Hodgkin Lymphoma of Gallbladder Presenting as Cholecystitis.

Paranasal Sinus Lymphoma Presenting as an Orbital Mass

The paranasal sinuses are rare sites for origination of primary Non-Hodgkin’s lymphoma (NHL). Most often, primary paranasal lymphomas are diffuse large B-cell lymphomas (DLBCL) associated with a poor prognosis. This paper describes an uncommon case of ethmoid sinus lymphoma with lateral displacement of the left orbit and involvement of the nasal vault, skull base, and frontal sinuses. Excisional biopsy and flow cytometry findings favored the diagnosis of DLBCL, non-germinal center type. Moreover, CT showed an epigastric mass and enlarged lymph nodes along the left iliac chain and spleen. Radiation therapy was immediately started and the patient achieved significant symptomatic relief after four treatments. Accurate diagnosis of extranodal NHL lymphomas through a thorough history, physical exam, imaging, and immunopathology is necessary to initiate prompt treatment of this rare, aggressive disease.

Sclerotic changes on plain X-ray led to early diagnosis and successful treatment of primary lymphoma of iliac bone.

Sir, A previously healthy 54-year-old woman presented to our hospital with a 3-month history of pelvic pain. There was no history of constitutional symptoms such as unintentional weight loss, fever, and loss of appetite. Clinical examination revealed pain on deep palpation of the right iliac region. There was no evidence of soft tissue swelling or redness in the site of patient's pain. The rest of physical exam was unremarkable. Laboratory investigations were within normal limits (erythrocytes sedimentation rate of 20 mm in the 1st h, negative C-reactive protein and white blood count of 4800 per μl). A plain radiograph of the pelvis showed slightly lytic-sclerotic changes of right iliac bone with indistinct margins, which at a shallow observation confused with bowel gases [Figure 1]. A computed tomography (CT) was performed and showed a slightly expansile lytic-sclerotic lesion with small soft tissue component in the right iliac bone [Figure 2]. The bone scan also revealed isolated uptake of technetium-99m in the right ilium [Figure 3]. According to the patient age, the differential diagnosis included metastasis, lymphoma, plasmacytoma, and chronic osteomyelitis. Figure 1 Radiograph of pelvis demonstrates lytic-sclerotic changes of right iliac bone Figure 2 Pelvic computed tomography image shows a slightly expansile lytic-sclerotic lesion in the right iliac bone with no extension to the sacroiliac joint Figure 3 Bone scan revealed isolated uptake of technetium-99 m in the right ilium The core needle biopsy result was consistent with the diagnosis of diffuse large B-cell nonHodgkin's lymphoma. There was not any other site of involvement on CT scan of the chest, abdomen, and pelvis. Positron emission tomography (PET) also showed no secondary absorption of fluorodeoxyglucose [Figure 4]. The diagnosis of primary nonHodgkin lymphoma of bone was made, and the patient was successfully treated with a combination of chemotherapy and radiotherapy. Figure 4 Positron emission tomography scan showed no secondary absorption of fluorodeoxyglucose NonHodgkin's lymphoma of bone is a rare entity which is limited to the long bones and axial skeleton, with the femur being the most common site of involvement. Primary involvement of iliac bone is extremely rare. There are no clinical or radiological characteristics which be diagnostic for primary bone lymphoma. The image findings in radiograph or CT scan are variable and nonspecific, range from near normal to diffuse permeative pattern.[1,2,3,4,5,6] A review article performed by Mulligan et al., revealed that the most common features on imaging studies are lytic (70%), including permeative or moth-eaten pattern, and mixed lytic and sclerotic density (28%). Periosteal reaction and soft tissue mass were reported in 58%, and 80-100% of cases, respectively, in this study.[2] Sequestrum formation considered as a radiological feature which may help to differentiate between plasmablastic lymphoma (PBL) and other conditions such as metastasis and myeloma. Sequestrum formation has been reported in 11-16% of PBL cases.[7] Cross-sectional imaging studies are useful adjuncts to conventional radiography. Extensive involvement of bone marrow cavity with a surrounding soft tissue component and without extensive cortical destruction is a pattern, which is reported only in PBL, Ewing sarcoma, and myeloma.[8] The key educational message of our case is that any change on plain X-ray is valuable and should not be overlooked. This case also reminds us the importance of considering primary nonHodgkin lymphoma as a valuable differential diagnosis in lytic and sclerotic lesions of iliac bone. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Primary Non-Hodgkins Lymphoma of Uterine Cervix: A Case Report of Two Patients.

Primary lymphoma of the female genital tract (FGT) is a rare disease and accounts for less than 1 % of extra nodal lymphomas in females. In a series of 147 primary genital tract non-Hodgkins lymphoma, ovarian lymphoma (59 %) was the commonest followed by uterine corpus (15.5 %), uterine cervix (11.5 %), vulva, (7.5 %), and vagina (6 %) [1]. Role of surgery in this disease is questionable. Combined modality approach using chemoradiation has been used by various authors, which is associated with good prognosis. We report two cases of uterine lymphoma who presented to our clinic with pain in abdomen. Neither patient had any history of bleeding per vaginum.

English

Bilateral primary non-Hodgkin's lymphoma of breast is a rare entity because incidence wise it comprises less than 10% of all primary breast lymphoma. Here a case of bilateral breast lump is reported and reviewed, who had bilateral axillary node involvement and which trucut biopsy showed evidence of primary non-Hodgkin's lymphoma and responded satisfactory to chemotherapy. KEYWORDS: Breast tumor; Bilateral; Primary Non-Hodgkin's Lymphoma. INTRODUCTION: Primary mammary involvement in NHL is rare and representing 0.4-0.7 % of all diagnosed cases of non-Hodgkin's lymphoma. (1) Less than 0.6% of all breast malignancy is primary breast lymphoma.(2) Breast lymphoma usually presents as a painless unilateral breast mass and bilateral disease is seen in less than 10 %.(3) Such lymphomas are mostly non-Hodgkin's type representing 70-90%.(1) Primary breast lymphomas exhibit a poor prognosis and its therapeutic management is controversial and till date not fully established. We report a bilateral primary lymphoma (non-Hodgkin's) of breast due to its rarity.

Osteolytic Thoracic Spinal Lesions at Presentation in Primary Non-Hodgkin's Lymphoma of the Bone

Osteolytic Thoracic Spinal Lesions at Presentation in Primary Non-Hodgkin's Lymphoma of the Bone

Diagnosis of Primary Follicular B-Cell Non-Hodgkin’s Lymphoma of the Spleen by EUS-Guided FNA

Diagnosis of Primary Follicular B-Cell Non-Hodgkin’s Lymphoma of the Spleen by EUS-Guided FNA

Primary extranodal non-Hodgkin's lymphoma: clinicopathological features, survival and treatment outcome in two cancer centers of southern Turkey.

The aim of this study was to assess the epidemiological and clinicopathological characteristics of primary extranodal non-Hodgkin's lymphoma (pENL) patients, focusing on treatment and survival outcome. Between October 2003 and March 2012, 802 patients with non-Hodgkin's lymphoma (NHL) were diagnosed and treated in two different cancer centers of Southern Turkey. pENL, constituted 12.4% (100/802) of all NHL studied during this period. Median age of the patients was 56 years (range 17-87 years) and the male: female distribution was 3:2. Eighty-five of 100 patients (85%) were in stage I/II, 9/100 (9%) in stage III, whereas 6/100 (6%) were in stage IV. Head and neck constituted the most common site (51/100, 51%), followed by gastrointestinal tract (GIL) (37/100, 37%), and cerebrum (CL) (5/100, 5%). Diffuse large B cell lymphoma (DLBCL) was the most common histological type, observed in 53% of patients, followed by marginal zone extranodal lymphoma (13%). Most of patients (76%) received a CHOP containing regimen. Complete remission (CR) were achieved in 71% of patients. The median follow-up duration of all patients was reported as 37.6 months (range, 0.8-165 months). This period was reported as 137.5 months (range, 117.5- 1578.6 months) in gastrointestinal lymphoma (GIL) patients, 119.0 months (range, 91.8-146.1 months) in head and neck lymphoma (HNL) patients, and 18.4 months (range, 12.6-24.1 months) in cerebral lymphoma (CL) patients. Head and neck, and the gastrointestinal tract were the two most common extranodal sites observed. Histologically DLBC accounted for the majority of cases. Most patients were on earlier stages, had low-low intermediate IPI scores and had a favorable prognosis.

Institutional review of primary non-hodgkin lymphoma of the female genital tract: a 33-year experience.

The aim of this is to provide an updated review of the literature and to report our institutional experience with this rare gynecologic malignancy. The medical records of patients with diagnosis of non-Hodgkin lymphoma of the female genital tract from 1980 to 2013 at the Yale-New Haven Hospital were reviewed retrospectively. Histological classification and staging were determined by the World Health Organization and Ann Arbor systems, respectively. Kaplan-Meier was used to calculate the survival. There were 36 patients with diagnosis of non-Hodgkin lymphoma of the female genital tract and followed for a median of 61 months (0-361 months). The median age of diagnosis was 44 years (19-87 years), and 76% (n = 28) were classified as stage IV.Of these, 4 patients were asymptomatic on presentation, and 13 were identified incidentally during surgery/radiography (n = 9), on prenatal ultrasound (n = 1), and on Papanicolaou test (n = 3). The location of the disease included the ovary (n = 6), uterine corpus and cervix (n= 9), vagina (n = 1), a pelvic mass (n = 7), isolated pelvic/para-aortic lymph nodes (n = 3), and/or multiple sites (n = 9). There were 6 cases that were concomitant with other gynecologic malignancies.Diffuse large B-cell lymphoma (n= 18) was the most common histologic type. A total of 28 patients underwent surgery. Combination chemotherapy was used in 34 patients, with concomitant radiation therapy in 7 and stem cell transplantation in 3. A total of 5 patients had recurrent disease.The overall median survival from the diagnosis of lymphoma was 70 months (0.3-361 months) with a 91% 1-year survival, 86% 5-year survival, and a 79% 10-year survival. Our report is the largest published single-institution experience of this disease. It demonstrates a more favorable prognosis and proposes that with early diagnosis and appropriate therapy, radical gynecologic surgery can be avoided.

Primary diffuse large B-cell non-Hodgkin lymphoma of the paranasal sinuses presenting as cavernous sinus syndrome.

Primary paranasal sinus lymphoma is an uncommon malignancy that accounts for only 0.17% of all lymphomas and is often difficult to diagnose. Initially, tumors in this area cause very few symptoms and are at an advanced stage at the time of diagnosis. Ophthalmologic signs and symptoms occur early in the disease process because of close proximity of the orbit to the paranasal sinuses. We report on a case of primary malignant lymphoma of the sphenoid and ethmoid sinuses with involvement of the cavernous sinus, initially presenting with ophthalmologic signs and symptoms in association with cavernous sinus syndrome.

Bilateral primary adrenal non-hodgkin lymphoma.

Bilateral primary adrenal non-hodgkin lymphoma.

Primary Non-Hodgkin Lymphoma of the Ovary

Primary Non-Hodgkin Lymphoma of the Ovary