We report a 25-year-old young man presenting with cognitive decline, pancerebellar features, spastic quadriparesis, bilateral cataract (operated) and tendo-Achilles swelling (xanthoma). The CT of the head showed bilateral cerebellar hypodensities. There were bilateral cerebellar hypointensities involving dentate nuclei on T1-weighted images with corresponding hyperintensities on T2-weighted MRI. Additionally, an interesting MRI finding—‘hot cross bun’ appearance was seen in pons which has not been reported in the literature so far. Biopsy from tendo-Achilles confirmed xanthoma. He was treated with chenodeoxycholic acid following which he showed improvement in cognition and weakness.
Sentinel lymph node biopsy has been proposed as an alternative to up-front elective neck dissection (END) for determination of pathologic nodal status in patients undergoing surgical treatment for oral cavity squamous cell carcinoma (OSCC) with clinically negative neck (cN0). Sentinel lymph node biopsy using current standard tracer agents and imaging adjuncts such as radiolabeled sulfur-colloid and planar lymphoscintigraphy (LS), however, is associated with several drawbacks.
To assess the preliminary utility of technetium Tc 99m(99mTc)-tilmanocept, a novel molecular imaging agent for sentinel lymph node (SLN) mapping, in OSCC.
DESIGN, SETTING, AND PARTICIPANTS
Prospective, nonrandomized, single-arm, part of an ongoing phase 3 clinical trial. Patients had previously untreated, clinically and radiographically node-negative OSCC (T1-4aN0M0) at an academic tertiary referral center.
Patients received a single dose of 50 μg 99mTc-tilmanocept injected peritumorally followed by dynamic planar LS and fused single-photon emission computed tomography/computed tomography (SPECT/CT) prior to surgery. Surgical intervention consisted of excision of the primary tumor and radioguided SLN dissection followed by planned END. The excised lymph nodes (SLNs and non-SLNs) underwent histopathologic evaluation for presence of metastatic disease.
MAIN OUTCOMES AND MEASURES
False-negative rate and negative predictive value of SLNB using 99mTc-tilmanocept and comparison of planar LS with SPECT/CT in SLN localization.
Twelve of 20 patients (60%) had metastatic neck disease on pathologic examination. All 12 had at least 1 SLN positive for metastases. No patients had a positive END node who did not have at least 1 positive SLN. These data yield a false-negative rate of 0% and negative predictive value of 100% using 99mTc-tilmanocept in this setting. Dynamic planar LS and SPECT/CT revealed a mean (range) number of hot spots per patient of 2.9 (1-7) and 3.7 (1-12), respectively. Compared with planar LS, SPECT/CT identified additional putative SLNs in 11 of 20 cases (55%).
CONCLUSIONS AND RELEVANCE
The high negative predictive value and low false-negative rate in identification of occult metastases shows 99mTc-tilmanocept to be a promising agent in SLN identification in patients with OSCC. Use of SPECT/CT improves preoperative SLN localization including delineation of SLN locations near the primary tumor when compared with planar LS imaging.
clinicaltrials.gov Identifier: NCT00911326
The purpose of this study was to determine factors that impact recurrence and long-term survival of head and neck adenoid cystic carcinoma (ACC).
We conducted a retrospective review of 87 patients with head and neck ACC who were evaluated between 1992 and 2009. Staining for Ki-67, p53, α-estrogen receptor (αER), and progesterone receptor (PR) was performed.
Forty men (46%) and 47 women (54%) were included in this study. Median follow-up for patients was 98 months. Five-year recurrence-free and overall survival (OS) rates were 56% and 81%, respectively. Ki-67 and p53 expression was observed in 5 (6%) and 2 (2%) patients, respectively. αER and PR were all negative. The most important determinants of disease-free survival (DFS) were perineural invasion (PNI; p = .001) and female sex (p = .027). Disease site (major vs minor salivary gland) was the only predictor of worse OS on multivariate analysis.
Perineural invasion, female sex, and disease site were the most consistent predictors of poor outcome in head and neck ACC.
adenoid cystic carcinomas; head and neck; prognostic factors; disease-free survival; overall survival
Background. We investigated a novel technique, intraoperative 99 mTc-Sestamibi (MIBI) imaging (neck and excised specimen (ES)), using a large field-of-view portable gamma camera (LFOVGC), for expediting confirmation of MIBI-avid parathyroid adenoma removal. Methods. Twenty patients with MIBI-avid parathyroid adenomas were preoperatively administered MIBI and intraoperatively imaged prior to incision (neck) and immediately following resection (neck and/or ES). Preoperative and intraoperative serum parathyroid hormone monitoring (IOPTH) and pathology (path) were also performed. Results. MIBI neck activity was absent and specimen activity was present in 13/20 with imaging after initial ES removal. In the remaining 7/20 cases, residual neck activity and/or absent ES activity prompted excision of additional tissue, ultimately leading to complete hyperfunctioning tissue excision. Postexcision LFOVGC ES imaging confirmed parathyroid adenoma resection 100% when postresection imaging qualitatively had activity (ES) and/or no activity (neck). The mean ± SEM time saving using intraoperative LFOVGC data to confirm resection versus first IOPTH or path result would have been 22.0 ± 2 minutes (specimen imaging) and 26.0 ± 3 minutes (neck imaging). Conclusion. Utilization of a novel real-time intraoperative LFOVGC imaging approach can provide confirmation of MIBI-avid parathyroid adenoma removal appreciably faster than IOPTH and/or path and may provide a valuable adjunct to parathyroid surgery.
To report long-term, health-related quality of life (HRQOL) outcomes in patients treated with transoral robotic surgery (TORS).
Prospective clinical study on functional and HRQOL outcomes in TORS.
University tertiary care facility.
Patients who underwent TORS at The Ohio State University Medical Center.
All patients undergoing TORS were asked to complete the Head and Neck Cancer Inventory before treatment, and at 3 weeks, 3, 6, and 12 months postoperatively. Demographic, intraoperative, clinicopathological, and follow-up functional data were collected for each patient.
Sixty four patients who underwent TORS were enrolled with a median age of 56.8years. A total of 113 TORS procedures were performed. Mean follow up time was 16.3 ± 7.49 months (range 6 to 33). Majority of TORS were performed for squamous cell carcinoma (88%). No patients experienced immediate postoperative complications, with all of the patients tolerating an oral diet without any airway compromise on the day of surgery. There was a decrease from baseline in the speech, eating, aesthetic, social, and overall QOL domains immediately after treatment. At the one year follow up, the HRQOL scores in the aesthetic, social, and overall QOL domains were near baseline. Patients with malignant lesions had significantly lower postoperative HRQOL scores in the speech, eating, social, and overall QOL domains (p<.05). Forty nine patients (77%) underwent adjuvant radiation therapy (RT), and 61% had chemoradiation (CRT) therapy. Patients who underwent adjuvant XRT or CRT had lower postoperative scores in the eating, social and overall QOL domains, compared to those who did not (p<.05).
TORS is a safe procedure with good functional and HRQOL outcomes. Patients who undergo TORS for malignancies and receive adjuvant therapy tend to have lower HRQOL outcomes. TORS is a promising future alternative surgical treatment for laryngopharyngeal tumors.
Transoral robotic surgery; health-related quality of life; head and neck cancer
To report long-term, health-related quality-of-life (HRQOL) outcomes in patients treated with transoral robotic surgery (TORS).
Prospective, longitudinal, clinical study on functional and HRQOL outcomes in TORS.
University tertiary care facility.
Subjects and Methods
Patients who underwent TORS were asked to complete a Head and Neck Cancer Inventory before treatment and at 3 weeks and 3, 6, and 12 months postoperatively. Demographic, clinicopathological, and follow-up data were collected.
Sixty-four patients who underwent TORS were enrolled. A total of 113 TORS procedures were performed. The mean follow-up time was 16.3 ± 7.49 months. The HRQOL was assessed at 3 weeks and at 3, 6, and 12 months, with a response rate of 78%, 44%, 41%, and 28%, respectively. TORS was performed most frequently for squamous cell carcinoma (88%). There was a decrease from baseline in the speech, eating, aesthetic, social, and overall QOL domains immediately after treatment. At the 1-year follow-up, the HRQOL scores in the aesthetic, social, and overall QOL domains were in the high domain. Patients with malignant lesions had significantly lower postoperative HRQOL scores in the speech, eating, social, and overall QOL domains (P < .05). Patients who underwent adjuvant radiation therapy or chemotherapy and radiation therapy had lower postoperative scores in the eating, social, and overall QOL domains (P < .05).
The preliminary data show that patients who undergo TORS for malignancies and receive adjuvant therapy tend to have lower HRQOL outcomes. TORS is a promising, minimally invasive, endoscopic alternative surgical treatment of laryngopharyngeal tumors.
transoral robotic surgery; health-related quality of life; head and neck cancer
The management of carcinoma of unknown primary (CUP) is one of the challenging conditions in head and neck oncologic surgery. Despite various diagnostic tools, the primary tumor site in more than half of cases remains unidentified. The purpose of this study was to assess the feasibility and efficiency of utilizing transoral robotic surgery (TORS) for the diagnosis and treatment of CUP in the head and neck.
In this prospective, single-institutional, clinical TORS trial, 22 of 181 patients were treated for CUP between 2008 and 2012.
Among all those 22 patients, primary tumor site identification and complete tumor removal was achieved in 17 patients (77.3%) with TORS. Tonsil (59.1%) and base of tongue (18.1%) were identified as the most common tumor locations.
Together with panendoscopy, directed biopsies, and positron emission tomography (PET)/CT, TORS is a valuable option in the identification and treatment of primary tumor sites.
carcinoma of unknown primary; transoral robotic surgery; daVinci
To determine speech, eating, aesthetics, social disruption, and overall quality-of-life outcomes over a year period in patients who underwent transoral robotic surgery as part of carcinoma of unknown primary diagnosis and treatment.
Observational prospective study.
Twenty-two patients who underwent transoral robotic surgery for the management of carcinoma of unknown primary were included. Patients prospectively completed the Head and Neck Cancer Inventory during a preoperative visit, and at 3-week, 3-month, 6-month, and 12-month postoperative visits. Patients’ demographic, pathological, and follow-up information were also collected.
The mean follow-up time was 19.8 months. There were overall declines in all quality of life scores during treatment period, which was followed by a continuous recovery. The scores immediately after transoral robotic surgery (3 weeks) were significantly higher than the scores after conclusion of adjuvant therapy (3 months) in multiple domains (P <.05) and the 6-month scores in speech (P = .02) and eating (P = .008) domains. All scores, except for eating (P = .01) returned to pre-treatment levels at 1 year. Patients with detected primaries displayed similar quality-of-life scores compared to patients with occult primaries. Human papillomavirus status and type of adjuvant treatment had no significant impact on quality of life.
Transoral robotic surgery is a promising, minimally invasive procedure for the surgical management of carcinoma of unknown primary. Patients maintain high functional and quality-of-life status at 1 year after surgery.
Transoral robotic surgery; carcinoma of unknown primary; quality of life; transoral robotic surgery; carcinoma of unknown primary; neck metastasis
Malignant peripheral nerve sheath tumors (MPNST) are rare spindle-cell sarcomas derived from Schwann cells or pluripotent cells of the neural crest accounting for less than 10 % of all soft tissue sarcomas. They arise from major or minor peripheral nerve fibers or their sheaths. The World Health Organization coined the term MPNST for tumors of neurogenic origin with similar biological behavior replacing all the previous heterogeneous and, often, confusing nomenclature including malignant schwannoma, malignant neurilemmoma, and neurofibrosarcoma. The retroperitoneum and the lower extremities are the most common sites, but MPNST may arise anywhere in the body. Its location in the retroperitoneum in a patient without neurofibromatosis is an exceedingly rare occurrence. Imaging is routinely performed to assess the extent of the disease and to plan surgical resection. Surgical resection is the first line of therapy, ideally with total removal of the tumor. Owing to a high risk of recurrence with incomplete resection, postoperative irradiation and chemotherapy are necessary; however, they are often used as adjuvant therapy even if the tumor is completely resected.
Malignant peripheral nerve sheath tumor; MPNST; Retroperitoneal sarcomas
Hyperpigmentation of gingiva becomes more pronounced if it is associated with “gummy smile.” Correction of gummy smile and depigmentation together are key to complete patient satisfaction. An 810 nm (1.5 W, pulsed) GaAlAs diode laser was used to achieve the desired results in a 22-year-old female patient. The 6-month follow-up results showed excellent color and contour of the gingiva. Mere depigmentation without correcting gummy smile may look cosmetically good but esthetically unacceptable. Diode laser was used as it is known to be an excellent tool as compared with other conventional surgical procedures in terms of patient and operator comfort.
Crown lengthening; depigmentation; diode laser; gummy smile
Colloid cysts are benign space-occupying lesions that account for 0.5-1.0% of brain tumors and arise from the velum interpositum or the choroid plexus of the third ventricle.
Material and Methods:
We are describing a modified surgical technique that combines the positive attributes of being minimalistic, while retaining the effectiveness of microsurgery. In all 20 consecutive symptomatic patients with a preoperative magnetic resonance imaging (MRI) diagnosis of colloid cyst who came to the senior author between 2008 and 2011 were included in the study. The patient was kept supine with the head positioned neutrally in the sagittal plane and neck flexed at 20°. The tube of a 5 ml plastic syringe having an external diameter of 13 mm and an internal diameter of 12.6 mm was cut toward the nozzle end to the appropriate length depending upon the cortical thickness measured on the preoperative MRI.
The average operative time was around 90 min with maximum of 120 min. None of the patients had seizures preoperatively or postoperatively and in all cases antiepileptic medication could be stopped after 3-6 month of surgery. Two patients had short-term memory impairment which returned to near normal by 1-year following surgery.
A volume of 5 ml plastic syringe port technique decreases the operative morbidity and operative time. The wider corridor of working makes the simultaneously maneuverability of two surgical instruments feasible enhancing safety and completeness of excision.
Approach; colloid cysts; single port; third ventricle
With the advancement of technologies there is more interest in the maintenance of the spine's biomechanical properties focusing on the preservation of the functional motion segment. In present article we describe our experience with 25 cases managed with artificial cervical discs with 28 Solis cage following cervical discectomy with a mean follow-up period of 7.5 year.
Materials and Methods:
All surgeries were performed by single surgeon from March 2004 to June 2005 with a follow-up till date. Patients with symptomatic single or multiple level diseases that had no prior cervical surgery were candidates for the study. Cohort demographics were comparable. Standardized clinical outcome measures and radiographic examinations were used at prescribed post-operative intervals to compare the treatment groups. Relief in radicular pain, cervical spine motion, and degenerative changes at follow-up were noted.
In a total 53 cases, the mean age in prosthesis group was 47 years (age range: 30-63 years) and mean age in cage group was 44 years (32-62 years). Mean hospital stay was 2.7 days in both the groups. At 4 weeks complete cervical movements could be achieved in 19 cases in artificial disc group. Maintenance of movement after 7.5 years was in 76% of these patients. Lordosis was maintained in all cases till date. There was no mortality or wound infection in our series.
We conclude that artificial cervical disc could be an alternative to fixed spinal fusion as it represents the most physiological substitute of disc. However, there is need for further studies to support the use of artificial cervical disc prosthesis.
Arthrodesis; cervical disc herniation; cervical arthroplasty; cervical prosthesis; prosthesis; spondylosis
While the majority of HPV+ oropharyngeal squamous cell carcinomas have a favorable prognosis, we search for markers of poor prognosis by carefully examining a subset of highly-aggressive cases.
Seven patients with HPV+ oropharyngeal cancer who presented with non-pulmonary distant metastasis or developed distant metastasis post-treatment were identified. Eight control cases were chosen which responded well to treatment. Pathology and radiological studies were reviewed and compared.
Two cases displayed a small cell carcinoma (SmCC) component upon pathologic review. Biomarker analysis revealed lower expression of NOTCH1 in the aggressive cohort in comparison to controls (p=0.04). Cases showed a predominance of clustering of lymph nodes, extracapsular spread and central tumor necrosis.
While most HPV-related oropharyngeal cancers display a positive prognosis, it is evident that there is a subset, which behave more aggressively. This early investigation identifies pathologic and radiologic features that may help to predict this behavior.
Head and neck cancer; Oropharyngeal squamous cell carcinoma; aggressive; HPV; human papillomavirus; neuroendocrine; small cell carcinoma; NOTCH1; p53; radiology
Background and Objectives:
The study was undertaken with the objective to provide data on the ABO and Rh(D) blood group distribution and gene frequency across India.
Materials and Methods:
A total of 10,000 healthy blood donors donating in blood banks situated in five different geographical regions of the country (North, South, East and Center) were included in the study. ABO and Rh (D) grouping was performed on all these samples. Data on the frequency of ABO and Rh(D) blood groups was reported in simple numbers and percentages.
The study showed that O was the most common blood group (37.12%) in the country closely followed by B at 32.26%, followed by A at 22.88% while AB was the least prevalent group at 7.74%. 94.61% of the donor population was Rh positive and the rest were Rh negative. Regional variations were observed in the distribution. Using the maximum likelihood method, the frequencies of the IA, IB and IO alleles were calculated and tested according to the Hardy Weinberg law of Equilibrium. The calculated gene frequencies are 0.1653 for IA (p), 0.2254 for IB (q) and 0.6093 for IO (r). In Indian Population, O (r) records the highest value followed by B (q) and A (p); O > B > A.
The study provides information about the relative distribution of various alleles in the Indian population both on a pan-India basis as well as region-wise. This vital information may be helpful in planning for future health challenges, particularly planning with regards to blood transfusion services.
ABO; blood group; rhesus
Acute severe bronchospasm is an emergency situation and sometimes these children may fail to respond to conventional treatment and deteriorate rapidly to respiratory failure requiring mechanical ventilation. We present a case of 2-year-old girl, who presented with severe bronchospasm resulting in respiratory failure not responding to conventional management including mechanical ventilation and was found to be H1N1 positive. She was treated with ketamine infusion, which led to prompt improvement in airway obstruction.
H1N1 infection; ketamine; wheezing; bronchospasm; acute severe asthma
Calcifying Fibrous Tumour; Retroperitoneal tumor; Childhood fibrous pseudotumor
Tarlov (perineural) cysts of the nerve roots are common and usually incidental findings during magnetic resonance imaging of the lumbosacral spine. There are only a few case reports where cervical symptomatic perineural cysts have been described in the literature. We report such a case where a high cervical perineural cyst was masquerading as a cervical spinal tumor.
Tarlov's cysts; Perineural cyst; Spinal cyst; spine; Cervical spine
Intracranial dermoids may gradually reach an enormous size before the onset of symptoms. Common clinical presentations of intracranial epidermoid include headache and seizures. We present a case of a 35-year female patient with giant middle fossa epidermoid that presented with Holmes’ tremor syndrome, and we review the relevant literature. To the best of our knowledge, such a presentation has not previously been described in the literature.
Epidermoid cyst; Movement disorders; Essential tremor; Intention tremor; Rubral tremor; Tremor
The incidence of spinal dysraphism has significantly decreased over the last few decades, all over the world; however, still the incidence is much higher in developing countries with poor socioeconomic status.
Materials and Methods:
The present study includes all patients managed for spinal dysraphism over a period of one year (January 2011-December 2011). Details including demographics, antenatal care history, site and type of lesion, neurological examination, imaging finding, associated congenital anomalies, management offered, and outcome were recorded.
A total of 27 children were operated for spinal dysraphism during the study period (17 males and 11 females). Median age was 120 days (age range, 1 day to 6 years). Mothers of 15 children did not seek any regular antenatal checkup and only 13 mothers received folic acid supplementation during pregnancy. Fourteen children were delivered at home and 13 were at hospital. The most common site was lumbosacral region (67.8%). Seven patients had rupture of the sac at the time of presentation, one child had local infection, and four patients had hydrocephalus (requiring shunt before surgical repair). Two patients developed hydrocephalus at follow up, needing shunt surgery. The mean hospital stay was 7 days (range, 5 days to 31 days; median, 10 days).
Spinal dysraphism is still a major public health problem in developing countries. Management of patients with spinal dysraphism is complex and needs close coordination between pediatrician, neurologist, neurosurgeon, and rehabilitation experts. A large number of factors influence the outcome.
Meningocele; myelomeningocele; spinal dysraphism
The optimization of a purely negative depletion, enrichment process for circulating tumor cells, CTC's, in the peripheral blood of Head and Neck cancer patients is presented. The enrichment process uses a red cell lysis step followed by immunomagnetic labeling, and subsequent depletion, of CD45 positive cells. A number of relevant variables are quantified, or attempted to be quantified, which control the performance of the enrichment process. Six different immunomagnetic labeling combinations were evaluated as well as the significant difference in performance with respect to the blood source: buffy coats purchased from the Red Cross, fresh, peripheral blood from normal donors, and fresh peripheral blood from human cancer patients. After optimization, the process is able to reduce the number of normal blood cells in a cancer patient's blood from 4.05 × 109 to 8.04 × 103 cells/ml and still recover, on average, 2.32 CTC per ml of blood. For all of the cancer patient blood samples tested in which CTC were detected (20 out of 26 patients) the average recovery of CTCs was 21.7 per ml of blood, with a range of 282 to 0.53 CTC per ml of blood. Unlike a majority of other published studies, this study focused on quantifying as many factors as possible to facilitate both the optimization of the process as well as provide information for future performance comparisons. The authors are not aware any other reported study which has achieved the performance reported here (a 5.76 log10) in a purely negative enrichment mode of operation. Such a mode of operation of an enrichment process provides significant flexibility in that it has no bias with respect to what attributes define a CTC; thereby allowing the researcher or clinician to use any maker they choose to define whether the final, enrich product contains CTC's or other cell type relevant to the specific question (i.e. does the CTC have predominately epithelia or mesenchymal characteristics?).
Circulating tumor cells; Immunomagnetic cell separation; Immunocytochemistry; RT-PCR; Squamous cell carcinoma of the head and neck (HNSCC)
Giant cell tumor (GCT) is a benign neoplasm but locally aggressive tumor that uncommonly involves the skull bone. We report a case of a 62-year-old male presented with increasing headache and diplopia. Investigations were suggestive of an expanding mass lesion of the clivus. Histopathology was suggestive of diagnosed with GCT with abundant histiocytes.
Clivus; giant cell tumor; histiocytes; skull
This phase I study determined the maximal-tolerated dose, dose-limiting toxicities, pharmacokinetics, and recommended dose of erlotinib with docetaxel.
Patients and methods
Twenty-eight patients with head and neck cancer were enrolled. Patients were orally given erlotinib (50 mg) daily plus 35 mg/m2 of docetaxel intravenously weekly × 3 every 4 weeks. Dose escalation of erlotinib was in 50-mg increments until toxicity. Pharmacokinetics were studied with LC–MS/MS, standard, and population pharmacokinetic methods.
Ninety-five courses were successfully given (median 3, range 1–6). The most frequent side effects were diarrhea, fatigue, skin rash, anemia, and hypoalbuminemia. Dose de-escalation for both erlotinib and docetaxel was due to skin rash, neutropenia and/or severe infection with docetaxel to 25 mg/m2 and erlotinib to starting dose of 50 mg and re-escalation of docetaxel to 35 mg/m2. Responses were observed in 4/26 evaluable patients (100 mg erlotinib). In 24 patients, the mean Cmax and AUC erlotinib values increased with dose and following cumulative dosing (days 7 and 8 vs. day1, p < 0.05). The CL/F (~7 L/h), V/F (~140 L), and t1/2 (~20 h) for erlotinib were similar to the reported. The mean AUC ratio of metabolite OSI-420 to erlotinib following repetitive dosing at 100 mg (+ or − docetaxel) showed a ~50% increase (p < 0.02), possibly suggesting self-enzyme induction. Population pharmacokinetic studies showed no significant covariate affecting erlotinib pharmacokinetics.
The combination of erlotinib and docetaxel was associated with significant toxicity, which limited the amount of administered erlotinib. Dosing for phase II trials was docetaxel 35 mg/m2 and erlotinib 50 mg. The reason for excessive toxicity is not clear, but not due to change in pharmacokinetics.
Erlotinib; Squamous cell carcinoma of the head and neck; OSI-774; Phase I
Paraquat (1, r-dimethyl-4,4’-bipyridium dichloride), a brown syrupy liquid is an effective herbicide that has low chronic toxicity because of its rapid deactivation on contact with soil. A high dose of paraquat or severe poisoning has a poor prognosis. At present there is no specific antidote to paraquat poisoning, hence the need to focus on prevention and in case of exposure or ingestion, aggressive decontamination to prevent further absorption. Although uncommon, paraquat ingestion can lead to severe and often fatal toxicity. However, despite its widespread availability, reports of this herbicide poisoning in India are uncommon.
Mortality; outcome; paraquat positioning