A 55-Year-Old Woman With Frequent Pulmonary Exacerbations and Endobronchial Lesions.

CASE PRESENTATION: A 55-year-old woman with COPD, heart failure with preserved ejection fraction (congestive heart failure), diabetes mellitus, and hypertension presented with baseline dyspnea at rest that had worsened over the last week. She reported associated runny nose, congestion, and cough productive of green sputum. She smoked six cigarettes per day and denied alcohol, drugs, or occupational exposure. She was admitted and initiated on treatment for acute exacerbation of COPD; however, her condition did not improve with steroid, ceftriaxone, and nebulized albuterol and budesonide treatments. She had been diagnosed with asthma and COPD without ever undergoing pulmonary function testing. She presented 11 times to the ED with six hospital admissions in the last 1.5 years for worsening dyspnea at rest, wheezing, and lower extremity edema deemed secondary to exacerbation of her COPD or congestive heart failure. She reported medication compliance, which included fluticasone-vilanterol, tiotropium bromide, and furosemide. She repeatedly demonstrated mild vascular congestion on imaging without hyperinflation, a normal to mildly elevated brain natriuretic peptide (<10 to 200 pg/mL), and dyspnea without hypoxia. She was treated normally for both COPD and congestive heart failure exacerbations simultaneously with methylprednisolone, albuterol, and furosemide with rapid improvement over the course of 1 to 2 days. No significant improvement was noted with steroid therapy, despite receiving them as an inpatient and outpatient. At the time of discharge, her symptoms would be at her baseline.

Experience with Esophageal Granular Cell Tumors: Clinical and Endoscopic Analysis of 22 Cases.

BACKGROUND: Esophageal granular cell tumors (GCTs) are rare tumors. Differences in reports on the clinical features of GCTs in the esophagus and some controversies about the diagnostic strategy for esophageal GCTs exist. OBJECTIVES: We aimed to investigate the clinical features and diagnosis of esophageal GCTs. Additionally, we sought to determine the prevalence of gastroesophageal reflux disease and reflux esophagitis in patients with esophageal GCTs. METHODS: We retrospectively studied the clinical features, endoscopic features, and management of 22 patients with esophageal GCTs. RESULTS: Esophageal GCTs were more common in men than in women with a ratio of 1.2:1 and were predominantly found in the distal esophagus. Ten patients with esophageal GCTs had regurgitation and/or heartburn symptoms, and eight patients were confirmed to have reflux esophagitis by endoscopy. All esophageal GCTs were protuberant lesions covered by normal esophageal epithelium. The endoscopic morphology of esophageal GCTs was diverse. On endoscopic ultrasonography, these tumors appeared as homogeneous or inhomogeneous hypoechoic lesions with clear borders originating from the submucosal or mucosal layer. Eleven patients underwent endoscopic forceps biopsy at the first endoscopy, and only six patients were correctly diagnosed by pathology. Nevertheless, the 18 lesions treated with endoscopic resection were all correctly diagnosed without complications, and no patients developed recurrence during the follow-up period. CONCLUSIONS: The occurrence of esophageal GCTs may be related to esophageal inflammation. As a method for obtaining an accurate pathological diagnosis and for treatment, endoscopic resection should be offered as the primary option for patients with esophageal GCTs.

Laryngeal granular cell tumour: a very rare diagnosis for a child presenting with hoarse voice in the UK.

We present a rare case of a paediatric laryngeal granular cell tumour (GCT) of the vocal cord. GCTs are rare clinical entities, thought to arise from Schwann cells. There are only a handful of paediatric laryngeal GCTs in the literature, and therefore, little is known regarding their natural history or preferred management strategies. In this case report, we discuss the incidence, aetiopathogenesis, presentation and management of a laryngeal GCT in an 11-year-old girl, who presented with dysphonia. We believe that this case will aid otolaryngologists in the management of paediatric patients with the common dysphonia.

Malignant granular cell tumor of the median nerve: a case report with a literature review of 157 cases.

Malignant granular cell tumors are an extremely rare, high-grade sarcoma with a schwannian phenotype and are composed of malignant granular cells with cytoplasmic lysosomal inclusion. To date, 157 cases of malignant granular cell tumors have been reported. We report the first case of a malignant granular cell tumor arising from the digital nerve to the median nerve in the palm, and we review the 157 previously reported cases and summarize the clinical profile, treatment, and outcome of this tumor. The median age, tumor size, and follow-up periods were 51 years, 6 cm, and 24 months respectively. With respect to the oncological result, 53 patients (33.8%) had no evidence for disease, 31 (19.7%) were alive with the disease, and 51 (32.5%) died because of the disease. Our case report indicates that rare malignant tumors can arise from the digital nerve to the median nerve in the palm, an anatomical site that is usually affected by benign lesions. Exhaustive discussions between surgeons and pathologists are necessary for the treatment of this rare malignant tumor.

Malignant granular cell tumor: Clinical features and long-term survival.

BACKGROUND: Malignant granular cell tumor GCT (mGCT) has not been well described. We sought to investigate associations between tumor characteristics, treatments and survival. METHODS: Patients diagnosed with mGCT years 1995-2014 were identified using the Surveillance, Epidemiology and End Results database. Descriptive statistics regarding tumor and treatment characteristics were calculated. Chi-square tests determined associations between tumor location and features. Survival analyses included Kaplan-Meier functions and Cox proportional hazard ratios (HR). RESULTS: Of 113 patients included, median age was 54 years and 77.0% were female. Frequent tumor sites included soft tissues (36.3%), ovary/testis (16.8%), and skin (11.5%). Median tumor size was 4.0 cm. Metastases to regional lymph nodes (12.5%) and distant sites (11.4%) occurred. Treatments included surgery (85.0%), radiotherapy (12.4%) and chemotherapy (8.9%). Overall five and 10-year cause-specific survival was 74.3% and 65.2%, respectively. Survival was worse for patients with tumors >5 cm compared to those with tumors ≤5 cm (HR = 34.03; 95% confidence interval [CI]: 2.57-450.17), and patients with metastasis (HR = 15.25; 95% CI: 1.19-195.72) compared with those without metastasis. Patients who underwent surgery had superior survival than those who did not (HR = 0.13; 95% CI: 0.05-0.34). CONCLUSIONS: Particular tumor features and treatments are associated with superior survival. This information may be used to more accurately estimate prognosis.

Multiple morphologically distinct cutaneous granular cell tumors occurring in a single patient.

Granular cell tumors (GCTs) typically are benign solitary tumors derived from Schwann cells. The tongue and skin are the most common sites of involvement; however, lesions also can develop in viscera such as the gastrointestinal tract. Multiple cutaneous GCTs in a single patient have been reported, with the lesions being described as subcutaneous papules, nodules, or verrucous nodules. We report the case of a patient who presented with several simultaneously occurring cutaneous GCTs with morphologically distinct clinical appearances ranging from subcutaneous nodules with no overlying epidermal alteration to exophytic moist nodules with eroded surfaces. Histopathology of several lesions was diagnostic of GCTs. This case illustrates the highly varied clinical presentation and morphology of cutaneous GCTs, even those occurring in a single patient. In addition to mimicking other benign neoplasms, GCTs may mimic other disease processes, including malignant lesions, infections, and inflammatory disorders. Skin biopsy generally is required for definitive diagnosis.

How Long Is Too Long? Trials and Tribulations of an Indolent Tumor.

A 31-year-old African American man presented for workup of a right hilar and tracheal mass. Stability of the mass when compared with a computed tomographic scan performed 3 years prior suggested an indolent process. On bronchoscopy, there were 2 separate although morphologically similar endobronchial lesions, one in the distal trachea and the second at the level of the right upper lobe bronchus. Biopsies of both lesions demonstrated granular cell tumors. Subsequent rigid bronchoscopy with ablation led to resolution of wheeze, decrease in dyspnea, and documented improvements in both ventilation and perfusion to the right lung. This case illustrates both a rare disease (multifocal endobronchial granular cell tumor) and the physiological impact of reducing large airway obstruction.

Malignant granular cell tumor of the foot-multimodality imaging findings and literature review.

We describe how the combination of imaging and histologic findings was essential in establishing a preoperative diagnosis of an extremely rare malignant granular cell tumor (GrCT) occurring in the lower extremity of a 17-year-old man. Magnetic resonance imaging demonstrated a large infiltrative tumor of heterogeneous intermediate signal intensity on both T1- and T2-weighted sequences. Subsequent computed tomography (CT) and fluorodeoxyglucose positron emission tomography CT scans of the patient revealed distant nodal and skeletal metastases.

[Granular cell tumor: report of a complicated vulvar localization of pulmonary metastases]. / Tumeur à cellules granuleuses : à propos d'une localisation vulvaire compliquée de métastases pulmonaires.

Granular cell tumour, or Abrikossoff's tumour, is a rare, ubiquitous tumour, with mostly benign evolution. The malignant forms represent 1-3% of the cases. The vulvar localization is exceptional. We report the case of a 28-year-old patient who presented a vulvar Abrikossoff's tumour. Initially considered benign, the tumour was treated by exclusive local surgery. Five months later, the tumour showed a malignant transformation. The tumour size was 14cm in its major axis with the presence of not systematized scattered mitoses, PS 100 was positive at 100% and Ki 67 to 20%. Because of malignant and economic excision of the neoplasm, the patient received adjuvant radiotherapy. After 20 months, the patient had a local recurrence and lung metastasis, supporting the diagnosis of malignancy. She received two chemotherapy regimens of 5-fluoro-uracil/cisplatin and pacliatxel/carboplatin without any response. Following the failure of chemotherapy, the patient received symptomatic medical treatment. This observation leads to advocate an aggressive surgical treatment and strict supervision of all granular cell tumours.

Cytogenetic and molecular genetic study on granular cell glioblastoma: a case report.

Granular cell astrocytoma is a rare infiltrative malignant glioma with prominent granular cell change. Granular cell astrocytomas are biologically aggressive compared with conventional infiltrating astrocytomas of similar grades, but their genetic alterations are poorly known. We report a case of granular cell glioblastoma and its genetic and molecular features. Histologically, the tumor not only showed features typical of granular cell astrocytoma but also demonstrated frequent mitoses, pseudopalisading necrosis, and vascular endothelial hyperplasia, compatible with glioblastoma. Array-based comparative genomic hybridization and focused molecular genetic analyses demonstrated gain of chromosome 7; losses of chromosome 1p, 8p, 9p, 10, 13q, and 22q; amplification of epidermal growth factor receptor; and homozygous deletion of CDKN2A as well as MGMT promoter methylation. However, neither isocitrate dehydrogenase 1 mutation nor codeletion of 1p/19q was found. Our results indicate that granular cell glioblastomas, despite having its peculiar granular cell changes, share common molecular genetic features with conventional glioblastoma, especially the classical subtype.

Tumor cutáneo de células granulares en la infancia: presentación de un caso / Childhood cutaneous granular cell tumor: case report

El tumor de células granulares (TCG) es una neoplasia benigna de diferenciación distintiva a la microscopia de luz caracterizada por la presencia de células con citoplasma eosinofílicoabundante y granular. Los informes en niños son extremadamente raros. Se presenta el caso de un paciente de sexo masculino, de 11años de edad, con una lesión nodular de seis meses de evolución, que aumentó progresivamente de tamaño, asintomática, localizada en la espalda. A la exploración se evidencia un nódulo oval, de 1,5 cm de diámetro, móvil, no adherido ni doloroso. El estudio histológico muestra lesión tumoral no encapsulada, formada por células de gran tamaño, de abundante citoplasma ocupado por gránulos eosinofílicos y núcleos de localización central. El estudio inmunohistoquímico señala positividad para S100. Se indica como tratamiento la resección quirúrgica local conservadora. Destacamos la necesidad de biopsiar nódulos solitarios ulcerados persistentes en el tiempo, para tener certeza diagnóstica y resaltamos la importancia de la histopatología, ya que el diagnóstico clínico es difícil y casi nunca se piensa en él, salvo que la lesión se presente en la lengua.

Granular cell tumors of the larynx: diagnosis and management.

In this case study, a retrospective review of chart and literature was done to discuss the presentation and treatment of granular cell tumors of the larynx. One patient was diagnosed and treated successfully with surgical resection of a laryngeal granular cell tumor. Excellent voice recovery was obtained and there is no evidence of recurrence 6 months after surgery. Granular cell tumors should be considered in the differential diagnosis of laryngeal masses, particularly in the posterior glottis.

Abrikossoff cell tumor of the oesophagus: a case report and review of the literature.

Abrikossoff tumours or granular cell tumours are rare and usually benign tumours. The oesophagus is a rare location (0.001% of all tumours). The macroscopic appearance is typical (yellow, firm, well circumscribed submucosal neoplasm-like with reduced vascular patterns) but due to the rarity of this lesion, it can be mistaken with other similar lesions. This is the report of a case of a 47-year-old female with a granular cell tumour of the distal oesophagus. In this case, the Abrikossoff tumour is clinically, radiologically en histologically benign. According to the fact that there is low evidence in the literature for removing such Lesions in patient without symptoms, it was decided to be conservative. In this paper we discuss the case with review of the Literature with emphasis on the diagnosis, evaluation and management.

Granular cell tumors of the esophagus: report of five cases and review of diagnostic and therapeutic techniques.

Granular cell tumors (GCT) of the esophagus are stromal lesions originating from the Schwann cells of the submucosal neuronal plexus. Although they are very infrequent, they constitute the second largest cause of non-epithelial tumors in the esophagus after leiomyomas. These tumors are generally benign, although a certain number of malignant, aggressive cases have been reported. Diagnosis requires that this possibility be ruled out before deciding on which course of therapeutic action to take as well as familiarization with the relevant indicators. GCT linked synchronically or metachronically to other malignant neoplasias of the esophagus have also been described, but the actual extent of this association is uncertain. This report describes five cases of GCT recently diagnosed as incidental findings following endoscopic exploration. All of these were benign and were treated conservatively. The article discusses new aspects relating to the diagnosis of these lesions and the role carried out by endoscopic ultrasonography in their characterization, both at preliminary diagnosis and monitoring levels. No standard therapeutic guidelines exist for the management of GCT, but endoscopic treatment without invading the muscularis propria layer would be used for symptomatic patients, creating histopathological doubts requiring research on the entire organ. Endoscopic therapeutic techniques are analyzed (resection with forceps or diathermy handles, yttrium-aluminum-garnet laser ablation, alcohol injection) in esophageal GCT, which have overtaken surgery in most cases due to their efficiency, greater safety and fewer complications.

[Endobronchial granular cell tumor - what approach to take]. / Tumor de células granulares endobrônquico - Como abordamos?

Granular cell tumor is a mesenchymal neoplasm almost always benign, with tendency to recurrence. Although it is more frequent in in the head and neck it has been described in almost all areas of the body. Its occurrence in the lung is extremely rare. The authors describe two cases of endobronchial granular cell tumours, discuss the particularities of this pathology as well as the treatment options, with particular attention to the use of endobronchial excision and criotherapy.