Outcomes of cardiothoracic surgery in women with Turner syndrome.

Background: To describe short- and mid-term surgical outcomes of patients with Turner syndrome (TS) after cardiovascular interventions. Methods: All individuals >12 years of age at the time of surgical repair for cardiovascular disease (valve or coarctation repairs, aortic disease, aortic dissection) from 2002 to 2022 were eligible. The primary endpoint was complications or death within 30 days of intervention. Secondary outcomes included late complications and reinterventions within six months. Combined data from the University of Texas Health Science Center at Houston and the Turner Syndrome Society of the United States were included in the analysis. Results: We identified 22 patients who met the inclusion criterion. The median age was 46 years (range, 21-75 years), with 86% having estrogen replacement therapy. The most common medical condition was hypertension (77%), followed by hypothyroidism (59%). The most frequent indication for surgery was aortic root or ascending aortic aneurysms (68%), followed by symptomatic aortic stenosis in patients with bicuspid aortic valve (64%), coarctation of aorta (45%), and acute aortic dissection (18%). Respiratory complications were the most common (68%). Pleural effusions were the most frequent found sign on imaging studies (68%). Thoracentesis, or chest tube placement, was required in 33% (5/15). Respiratory failure requiring specific support with high flow oxygen and/or thoracentesis occurred in 36% (8/22). Conclusions: Patients with TS may be at an increased risk for postoperative complications after aortic surgery. Bicuspid aortic valve (59%) and coarctation of the aorta (45%) were the most common congenital malformations among our study group. Our study showed that respiratory complications were the most common, with pleural effusions being the most common post-surgery complication.

Second malignancies in patients with Hodgkin's Lymphoma: Half a century of experience.

Purpose: Therapeutic improvements for Hodgkin's Lymphoma (HL) has resulted in excellent survival outcomes. Thus, patients are increasing susceptible to developing secondary malignancy (SM) a feared iatrogenic complication. Materials & Methods: We evaluated the SM risk in a cohort of patients with HL treated over a 50-year period. In total, 1653 patients were treated for HL from 1956 to 2009 at a tertiary-cancer-center. A cumulative incidence function was used to quantify SM risk and the Fine and Gray competing risk model was used to identify disease and treatment related correlates. Results: Two-hundred-ninety patients (19%) developed SMs. Paradoxically, SM risk was higher in the modern era with 20-year cumulative incidence rates of 11.1%, 11.9%, 17% and 21.8%, for patients treated <1970, 1971-1986, 1986-1995 and 1996-2009, respectively. We hypothesized that the disproportionately high rate of early deaths in the early era may skew the assessment of SM risks, a much-delayed event. When the analysis was restricted to patients with early-stage favorable HL treated >1980, we found a reversal of the trend, especially on the risk of solid tumor, with a hazard ratio of 0.57 (p = 0.0651) in patients treated after 1996. Conclusion: Our findings highlight the limitations of comparing the risk of a late event between groups with disparate rates of early deaths, despite the use of a competing risk model. When partially corrected for, patients treated in the more recent time period experienced a lower solid tumor risk.

Benefit of consolidative radiation therapy for primary bone diffuse large B-cell lymphoma.

PURPOSE: Outcomes for patients with diffuse large B-cell lymphoma (DLBCL) differ according to the site of presentation. With effective chemotherapy, the need for consolidative radiation therapy (RT) is controversial. We investigated the influence of primary bone presentation and receipt of consolidative RT on progression-free survival (PFS) and overall survival (OS) in patients with DLBCL. METHODS AND MATERIALS: We identified 102 patients with primary bone DLBCL treated consecutively from 1988 through 2013 and extracted clinical, pathologic, and treatment characteristics from the medical records. Survival outcomes were calculated by the Kaplan-Meier method, with factors affecting survival determined by log-rank tests. Univariate and multivariate analyses were done with a Cox regression model. RESULTS: The median age was 55 years (range, 16-87 years). The most common site of presentation was in the long bones. Sixty-five patients (63%) received R-CHOP-based chemotherapy, and 74 (72%) received rituximab. RT was given to 67 patients (66%), 47 with stage I to II and 20 with stage III to IV disease. The median RT dose was 44 Gy (range, 24.5-50 Gy). At a median follow-up time of 82 months, the 5-year PFS and OS rates were 80% and 82%, respectively. Receipt of RT was associated with improved 5-year PFS (88% RT vs 63% no RT, P=.0069) and OS (91% vs 68%, P=.0064). On multivariate analysis, the addition of RT significantly improved PFS (hazard ratio [HR] = 0.14, P=.014) with a trend toward an OS benefit (HR=0.30, P=.053). No significant difference in PFS or OS was found between patients treated with 30 to 35 Gy versus ≥ 36 Gy (P=.71 PFS and P=.31 OS). CONCLUSION: Patients with primary bone lymphoma treated with standard chemotherapy followed by RT can have excellent outcomes. The use of consolidative RT was associated with significant benefits in both PFS and OS.

Comprehensive craniospinal radiation for controlling central nervous system leukemia.

PURPOSE: To determine the benefit of radiation therapy (RT) in resolution of neurologic symptoms and deficits and whether the type of RT fields influences central nervous system (CNS) control in adults with CNS leukemia. METHODS AND MATERIALS: A total of 163 adults from 1996 to 2012 were retrospectively analyzed. Potential associations between use of radiation and outcome were investigated by univariate and multivariate analysis. RESULTS: The median survival time was 3.8 months after RT. Common presenting symptoms were headache in 79 patients (49%), cranial nerve VII deficit in 46 (28%), and cranial nerve II deficit in 44 (27%). RT was delivered to the base of skull in 48 patients (29%), to the whole brain (WB) in 67 (41%), and to the craniospinal axis (CS) in 48 (29%). Among 149 patients with a total of 233 deficits, resolution was observed in 34 deficits (15%), improvement in 126 deficits (54%), stability in 34 deficits (15%), and progression in 39 deficits (17%). The 12-month CNS progression-free survival was 77% among those receiving CS/WB and 51% among those receiving base of skull RT (P=.02). On multivariate analysis, patients who did not undergo stem cell transplantation after RT and base of skull RT were associated with worse CNS progression-free survival. CONCLUSIONS: Improvement or resolution of symptoms occurred in two thirds of deficits after RT. Comprehensive radiation to the WB or CS seems to offer a better outcome, especially in isolated CNS involvement.

Radiation therapy is an effective modality in the treatment of mantle cell lymphoma, even in heavily pretreated patients.

INTRODUCTION: Mantle cell lymphoma has an aggressive clinical course and continuous relapse pattern with a median survival of 3 to 7 years. Multiple courses of chemotherapy are the basis of treatment. Radiotherapy is underutilized in this disease. We undertook this study to assess the role of radiation therapy. MATERIALS AND METHODS: A total of 41 consecutive patients with mantle cell lymphoma diagnosed from December, 1999 to January, 2010 who received radiation therapy were reviewed retrospectively. The main endpoint was in-field lymphoma response at each irradiated disease site. RESULTS: There were 39 evaluable patients (68 symptomatic sites). Sites treated included: nodal stations (n = 31), soft tissue (n = 13), mucosal sites (n = 11), central nervous system (n = 10), gastrointestinal tract (n = 2), and bone (n = 1). Median maximum tumor size at presentation was 3.5 cm (range, 1.3 cm-9.6 cm). The median dose of radiation was 30.6 Gy (range 18-40 Gy). Median follow-up post radiation per site was 12.3 months (range, 0.6-80.9 months). Response to treatment was complete in 47 sites (69.1%), partial in 16 sites (23.5%), and 5 sites (7.4%) had stable disease. In 9 (13.2%) sites local relapse occurred (median 7 months; range 2-21). The mean size of lymphoma at time of RT correlated with relapse, with tumors with local relapse larger than those without a local relapse (P = .005). CONCLUSIONS: Our data add to accumulating evidence that mantle cell lymphoma is a radio-sensitive disease with excellent responses to relatively low radiation doses, even in patients with chemo-refractory disease.

Dosimetric advantages of a "butterfly" technique for intensity-modulated radiation therapy for young female patients with mediastinal Hodgkin's lymphoma.

PURPOSE: High cure rates for Hodgkin's lymphoma must be balanced with long-term treatment-related toxicity. Here we report an intensity-modulated radiation therapy (IMRT) technique that achieves adequate target coverage for mediastinal disease while minimizing high- and low-dose exposure of critical organs. METHODS AND MATERIALS: Treatment plans for IMRT and conventional anteroposterior-posteroanterior (AP-PA) techniques, with comparable coverage of the planning target volume (PTV), were generated for 9 female patients with mediastinal Hodgkin's lymphoma assuming use of inclined positioning, daily breath-hold, and CT-on-rails verification. Our "butterfly" IMRT beam arrangement involved anterior beams of 300°-30° and posterior beams of 160°-210°. Percentages of normal structures receiving 30 Gy (V30), 20 Gy (V20), and 5 Gy (V5) were tabulated for the right and left breasts, total lung, heart, left and right ventricles, left anterior descending coronary artery (LAD), and spinal cord. Differences in each variable, conformity index, homogeneity index, and V107% between the two techniques were calculated (IMRT minus conventional). RESULTS: Use of IMRT generally reduced the V30 and V20 to critical structures: -1.4% and +0.1% to the right breast, -1.7% and -0.9% to the left breast, -14.6% and -7.7% to the total lung, -12.2% and -10.5% to the heart, -2.4% and -14.2% to the left ventricle, -16.4% and -8.4% to the right ventricle, -7.0% and -14.2% to the LAD, and -52.2% and -13.4% to the spinal cord. Differences in V5 were +6.2% for right breast, +2.8% for left breast, +12.9% for total lung, -3.5% for heart, -8.2% for left ventricle, -1.5% for right ventricle, +0.1% for LAD, and -0.1% for spinal cord. Use of IMRT significantly reduced the volume of tissue receiving 107% of the dose (mean 754 cm3 reduction). CONCLUSIONS: This butterfly technique for IMRT avoids excess exposure of heart, breast, lung, and spinal cord to doses of 30 or 20 Gy; mildly increases V5 to the breasts; and decreases the V107%.

Positron emission tomography/computed tomography findings during therapy predict outcome in patients with diffuse large B-cell lymphoma treated with chemotherapy alone but not in those who receive consolidation radiation.

PURPOSE: To assess the value of mid-therapy positron emission tomography (PET) findings for predicting survival and disease progression in patients with diffuse large B-cell lymphoma, considering type of therapy (chemotherapy with or without radiation therapy). METHODS AND MATERIALS: We retrospectively evaluated 294 patients with histologically confirmed diffuse large B-cell lymphoma with respect to age, sex, disease stage, International Prognostic Index score, mid-therapy PET findings (positive or negative), and disease status after therapy and at last follow-up. Overall survival (OS) and progression-free survival (PFS) were compared according to mid-therapy PET findings. RESULTS: Of the 294 patients, 163 (55%) were male, 144 (49%) were age >61 years, 110 (37%) had stage I or II disease, 219 (74%) had International Prognostic Index score ≤2, 216 (73%) received ≥6 cycles of rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone, and 88 (30%) received consolidation radiation therapy. Five-year PFS and OS rates were associated with mid-therapy PET status: PFS was 78% for those with PET-negative (PET-) disease versus 63% for PET-positive (PET+) disease (P=.024), and OS was 82% for PET- versus 62% for PET+ (P<.002). These associations held true for patients who received chemotherapy only (PFS 71% for PET- vs 52% PET+ [P=.012], OS 78% for PET- and 51% for PET+ [P=.0055]) but not for those who received consolidation radiation therapy (PFS 84% PET- vs 81% PET+ [P=.88]; OS 90% PET- vs 81% PET+ [P=.39]). CONCLUSION: Mid-therapy PET can predict patient outcome, but the use of consolidation radiation therapy may negate the significance of mid-therapy findings.

Clinical implications of positron emission tomography-negative residual computed tomography masses after chemotherapy for diffuse large B-cell lymphoma.

Response to primary treatment in diffuse large B-cell lymphoma (DLBCL) is highly predictive of long-term outcome. We evaluated the value of computed tomography (CT) findings relative to positron emission tomography (PET) findings, after the completion of chemotherapy. We retrospectively reviewed records from 491 patients with DLBCL at M. D. Anderson in 2001-2007; 22 patients were excluded for uncertain pathology and 169 for having received consolidative radiation, leaving 300 patients for the present analysis (median age, 61 years; 53% men, 47% women; 27% stage I-II, 73% stage III-IV; 73% completed 6-8 cycles of doxorubicin-based therapy). Factors associated with outcome on univariate analysis were response according to PET/CT and CT (p < 0.0001 for overall survival [OS], disease-specific survival [DSS] and progression-free survival [PFS]); number of chemotherapy cycles received (p < 0.0001 OS, p < 0.0001 DSS, p < 0.002 PFS); the combined presence of Ki-67 > 50%, PET SUV ≥ 13 and bulky (> 5 cm) disease (p = 0.005 OS, p = 0.001 DSS, p = 0.001 PFS); and International Prognostic Index (IPI) score (p = 0.004 OS, p = 0.005 DSS, p = 0.004 PFS). On multivariate analysis, PET/CT-negative, CT residual mass (> 2 cm) significantly influenced OS, DSS and PFS (p < 0.0001). The presence of a residual mass >2 cm on CT, coupled with negative findings on PET/CT, has prognostic value in DLBCL.

Cytogenetic profile of patients with acute myeloid leukemia and central nervous system disease.

BACKGROUND: Acute myeloid leukemia (AML) infrequently involves the central nervous system (CNS). This study was undertaken in patients with AML to determine whether cytogenetic findings predict CNS involvement. METHODS: The medical records of 1354 patients with AML who were treated at The University of Texas MD Anderson Cancer Center between January 2000 and December 2008 were reviewed. Forty patients (3%) had CNS involvement at time of presentation or disease recurrence, of whom 37 had conventional cytogenetics performed on bone marrow aspirate material. Demographics, treatment, and status at last follow-up were collected. RESULTS: Eleven patients (30%) had a diploid karyotype, and 14 patients (38%) had complex cytogenetics. Only 5 of the 40 patients had CNS disease at diagnosis, and the remaining patients had CNS disease at relapse. Patients who developed CNS disease were younger (P = .019), had a higher white blood cell (WBC) count at diagnosis (P = .001), had higher lactate dehydrogenase level (LDH) levels (P < .0001), and had higher percentages peripheral blast cells (P = .024) at diagnosis compared with the rest of the population. In addition, patients with CNS disease had higher rates of chromosome 16 inversion (P < .001), chromosome 11 abnormality (P = .005), and trisomy 8 (P = .02) and had a tendency toward complex cytogenetics (P = .2) compared with the control group (patients who had AML with no CNS involvement). CONCLUSIONS: Patients with AML and CNS disease often had higher LDH levels and WBC counts at diagnosis, and they often presented with chromosome 16 inversion and chromosome 11 abnormalities. The current study indicated that the overall survival of patients with AML who had CNS involvement is poor.

Consolidative radiation therapy for stage III Hodgkin lymphoma in patients who achieve complete response after ABVD chemotherapy.

OBJECTIVES: To examine the role of consolidation radiation therapy (RT) for patients with stage III Hodgkin lymphoma. METHODS: We retrospectively reviewed 118 patients with stage III Hodgkin lymphoma who were diagnosed and treated at the University of Texas M.D. Anderson Cancer Center from 1993 through 2006. We evaluated the influence of site and size of initial involvement and use of consolidative RT on survival and patterns of failure after complete response (CR) to ABVD chemotherapy (doxorubicin, bleomycin, vinblastine, and dacarbazine). RESULTS: After chemotherapy, 104 patients (88%) achieved CR; median follow-up time was 68 months (range, 8 to 190). Seventy-one patients (68%) received ≥6 cycles of ABVD, and 40 patients (38.5%) received consolidative RT. Comparing patients who received RT with those who did not, the 5-year, 10-year, and 15-year overall survival (OS) rates were 98%, 80%, and 80% versus 91%, 72%, and 29%, respectively (P=0.08). Disease-free survival (DFS) rates were 94%, 81%, 65% versus 78%, 45%, and 15%, respectively (P=0.04). On multivariate analysis, the presence of initial mediastinal involvement (P=0.001) and bulky head and neck disease (P=0.001) was associated with worse DFS; mediastinal RT was associated with improved DFS (P=0.003) and OS (P=0.029). Use of ≥6 cycles of ABVD was associated with improved OS (P=0.001). The pattern of failure analysis showed that most failures (23 of 28) occurred above the diaphragm. CONCLUSIONS: Consolidative RT after CR may benefit patients with initial disease above the diaphragm, whereas below-the-diaphragm disease seems to be well managed by chemotherapy alone.

Solitary plasmacytomas: outcome and prognostic factors after definitive radiation therapy.

BACKGROUND: The objective of this study was to review the outcome of patients with solitary plasmacytoma (SP) after definitive radiation therapy. METHODS: The authors retrospectively reviewed 84 patients with SP who were diagnosed and treated at The University of Texas MD Anderson Cancer Center during 1988 to 2008. The impact of tumor anatomic site, tumor size, and the presence of serum and urinary paraprotein at diagnosis was assessed on local control, survival, and the risk of developing multiple myeloma (MM). RESULTS: Fifty-nine patients (70%) had bone SP, and 25 patients (30%) had extramedullary SP. Serum paraprotein was present in 39 patients (46%). The median radiation dose was 45 grays (Gy) (range, 36-53.4 Gy). Local control was achieved in 77 patients (92%). Neither radiation dose nor tumor size predicted local control. The 5-year rate of progression to MM was 47% and was higher for patients with bone SP (56% vs 30% for extramedullary SP; P = .021), and patients who had serum paraprotein detected at diagnosis (60% vs 39%; P = .016). On univariate analysis, patients aged <60 years and men had higher rates of progression to MM, although the differences were not significant (P = .048 and P = .29, respectively). Multivariate analysis revealed that bone location and serum protein at diagnosis were associated statistically with progression to MM. The 5-year overall survival rate for the entire patient cohort was 78%, and no difference was observed between patients who had bone SP versus extramedullary SP (76% vs 85%, respectively; P = .274). CONCLUSIONS: The current results indicated that definitive radiation therapy for SP can provide excellent local control. Progression to MM remains the main problem and is more common among patients with bone SP and those who have serum paraprotein detected at diagnosis.

Incidence of gastric involvement in patients with nongastrointestinal extranodal marginal zone lymphoma.

BACKGROUND: The current study was conducted to determine the incidence of gastric involvement in patients presenting with extranodal marginal zone lymphoma (MZL) outside the gastrointestinal (GI) tract and to identify clinical or laboratory parameters that predict gastric involvement in such cases. METHODS: The records of 121 consecutive patients who presented with non-GI extranodal MZL and had undergone esophagogastroduodenoscopy (EGD) as part of their initial workup were retrospectively reviewed. The authors assessed the presence of occult gastric MZL in these patients and possible associations with demographic characteristics; anatomic site of initial presentation; Helicobacter pylori (H. pylori) infection; Zubrod score; International Prognostic Index (IPI); B symptoms; and serum lactate dehydrogenase, hemoglobin, albumin, and ß2-microglobulin levels. RESULTS: The median age at diagnosis of non-GI MZL was 59 years. The most common primary tumor sites were the salivary/parotid gland (32 patients), ocular adnexa (26 patients), skin (19 patients), and lung (17 patients). Twenty-two patients (18%) were found to have gastric involvement on EGD. Using logistic regression analysis, factors found to be associated with gastric involvement included: high IPI score (odds ratio [OR], 3.70; P = .03), female sex (OR, 6.50; P = .02), serum ß2-microglobulin level of ≥ 2.5 mg/L (OR, 3.69; P = .02), and involvement of the aerodigestive mucosal/glandular tissue (OR, 4.50; P = .004). On multivariate logistic analysis, aerodigestive mucosal/glandular sites, H. pylori infection, and an elevated ß2-microglobulin level were found to be associated with gastric involvement. CONCLUSIONS: Routine EGD is recommended for patients with non-GI MZL, particularly those with primary aerodigestive mucosal/glandular tissue involvement or those with a high IPI, female sex, elevated serum ß2-microglobulin level, or H. pylori infection regardless of the primary tumor site.

Successful treatment of a free-moving abdominal mass with radiation therapy guided by cone-beam computed tomography: a case report.

INTRODUCTION: Because tumors in the abdomen can change position, targeting these tumors for radiation therapy should be done with caution; use of daily image-guided radiation therapy is advised. CASE PRESENTATION: We report the case of a 72-year-old Caucasian man with recurrent mantle cell lymphoma who was referred for palliative radiation therapy for an abdominopelvic tumor. Computed tomography was used to generate images for radiation treatment planning. Comparison of those planning images with a positron emission tomography/computed tomography scan ordered during the planning period revealed that the tumor had moved from one side of the abdomen to the other during the three-day interval between scans. To account for this unusual tumor movement, we obtained a second set of planning computed tomography scans and used a Varian cone-beam computed tomography scanner with on-board imaging capability to target the tumor before each daily treatment session, leading to successful treatment and complete resolution of the mass. CONCLUSION: Abdominal masses associated with the mesentery should be considered highly mobile; thus, radiation therapy for such masses should be used with the utmost caution. Modern radiation therapy techniques offer the ability to verify the tumor location in real time and shift the treatment ports accordingly over the course of treatment.

Fertility drugs and the risk of breast cancer: a meta-analysis and review.

The risk of breast cancer has been associated with reproductive history. The purpose of this study was to determine the relationship between fertility drugs used in assisted reproductive procedures and the risk of breast cancer. We performed a literature search using the MEDLINE, the COCHRANE Library, and Scopus to identify studies linking breast cancer to fertility drugs. We excluded case series, case reports, and review articles from our analysis. The study populations included women who were treated for infertility with clomiphene, gonadotropins, gonadotropin-releasing hormones, or other unspecified fertility agents. We extracted information on study design, sample size, type of fertility drugs and number of treatment cycles, breast cancer incidence, and follow-up time from these studies. Eight case-control studies and fifteen cohort studies were included in the quantitative analyses. The Newcastle-Ottawa Quality Assessment Scales were used. Two investigators independently extracted study methods, sources of bias, and outcomes. We found that the risk of breast cancer was not significantly associated with fertility drug treatment. The follow-up periods were short in some of the studies analyzed in our study; however, we proceeded to test the trend in risk estimates across different durations of follow-up and found a trend for association using the nonparametric test; this was interpreted with caution in view of the lack of adjustment with other confounding factors. The current published data do not suggest higher risk of breast cancer in women who receive fertility treatment, but the lack of long-term follow up and the inherent weaknesses in some of the published studies have to be cautiously taken into account.

Benefit of consolidative radiation therapy in patients with diffuse large B-cell lymphoma treated with R-CHOP chemotherapy.

PURPOSE: The current standard therapy for patients with diffuse large B-cell lymphoma (DLBCL) is rituximab plus cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP). The role of consolidative radiation therapy (RT) in the setting of R-CHOP chemotherapy is not well reported. This retrospective analysis is an attempt to clarify this role. PATIENTS AND METHODS: Subjects were 469 patients with histologically confirmed DLBCL treated between January 2001 and December 2007. Variables including age, sex, Ann Arbor disease stage, bulky disease status, standardized uptake values (SUVs) on positron emission tomography (PET), International Prognostic Index (IPI), and Ki67 staining (proliferation). RESULTS: Of 469 patients, 190 (40.5%) had stage I or II disease and 279 (59.5%) had stage III or IV disease, 327 (70%) had at least six cycles of R-CHOP, and 142 (30.2%) had involved-field RT (dose, 30 to 39.6 Gy) after complete response to chemotherapy. Median follow-up was 36 months (range, 8 to 85 months). Multivariate analysis showed that RT (P < .0001), IPI score (P = .001), response to therapy (P = .001), use of six to eight cycles of R-CHOP (P < .001), and combined presence (P = .006) or absence (P = .025) of high Ki67, high PET SUV, and bulky disease influenced overall survival (OS) and progression-free survival (PFS). Matched-pair analyses of patients who received six to eight cycles of R-CHOP with stage I or II disease (44 pairs) and all stages (74 pairs) indicated that RT improved OS (hazard ratio [HR], 0.52 and 0.29, respectively) and PFS (HR, 0.45 and 0.24, respectively) compared with no RT. CONCLUSION: This study showed significant improvements in OS and PFS among patients who received consolidation RT after R-CHOP chemotherapy for DLBCL.

Hodgkin lymphoma involving extranodal and nodal head and neck sites: characteristics and outcomes.

BACKGROUND: Most Hodgkin lymphoma (HL) patients present with disease in nodal regions. However, in a small subset, disease develops in unique anatomic sites such as the head and neck area. This study aims to identify the characteristics and outcomes of patients who develop HL involving extranodal and nodal head and neck sites. METHODS: The authors searched The University of Texas M. D. Anderson Cancer Center's database for HL patients treated between 1967 and 2007 and included those with HL at head and neck sites. They reviewed the records for site of involvement, pathology, treatment, and survival. RESULTS: The authors identified 39 patients with extranodal and nodal HL of the head and neck. Five patients with lymphocyte predominant HL were excluded. Specifically, 10 of 34 patients had disease in the tonsils, 9 in the nasopharynx, 8 in the thyroid, 3 in the parotid, 2 in the adenoids, and 1 each in Waldeyer's ring and nasal antrum. Median age at diagnosis was 31.5 years, average age at diagnosis was 38 years, and 22 of 34 were male; 23 had stage I or II disease. Pathologically, 14 of 34 had the nodular sclerosis subtype, whereas 15 had mixed cellularity. Twenty-nine of 34 had nodal neck disease at presentation. Five of 34 received chemotherapy alone, 5 received radiation alone, and 24 received combination therapy. Twenty-one of 34 received 39.6 gray of external beam radiation. The most commonly used chemotherapy regimens were ABVD (doxorubicin, bleomycin, vinblastine, and dacarbazine) and MOPP (mechlorethamine, vincristine, procarbazine, and prednisone). At last follow-up, 85% were disease-free. CONCLUSIONS: HL of the head and neck is primarily diagnosed as early stage disease of men and of young to middle-aged individuals. Chemotherapy and primary/adjuvant radiotherapy offer excellent local and systemic control. The extent to which nodal disease is present in the neck does not alter outcomes when combined modality therapy is offered. Despite the unique anatomic location of these lesions, standard HL protocols work effectively to promote disease-free survival.