Pediatric granular cell tumor of the larynx: A case report and literature review.

An 8-year-old child was admitted to our ENT department for a year because of a hoarse voice. An endoscopic examination displayed that a cystic, solid lesion can be seen in the right subglottis. The lesion was removed using a CO2 laser under general anesthesia. Postoperative histopathology confirmed granular cell tumor (GCT), S-100(+), vimentin (+), and SOX-10(+). GCT, also known as the Abrikossoff tumor, is a rare benign tumor that rarely occurs in the larynx, particularly in children. This case report emphasizes that considerable attention should be given to the differential diagnosis of the laryngeal granulosa cell tumor. Given the recurrence risk of GCT, long-term postoperative follow-up is necessary.

Congenital Epulis: A Rare Head And Neck Tumor in the Newborn.

Congenital epulis is a rare tumor; when voluminous and ulcerated, appearance can generate concern. Nevertheless, it is a benign lesion that is easily treated with surgery. This is a retrospective study, with the presentation of 2 cases treated by the same team from 2016 to 2020 and a bibliographic review (PubMed and Elsiever). This surgical team is based in Buenos Aires, working in the private medical system. Congenital epulis is an infrequent, benign tumor affecting females predominantly, appearing at birth on the alveolar median ridge of the maxilla. Its pathogenesis is uncertain. Voluminous tumors interfere with feeding, but respiratory interference is exceptional. Histologically, this tumor is similar to the granular cell tumor but has different clinical characteristics and Periodic Acid Schiff stain. Prenatal diagnosis with ecographic or magnetic resonance imaging is ideal; if undiagnosed prenatally, clinical diagnosis is done based on typical features. Surgical excision is the treatment of choice.

Clinicopathological and immunohistochemical analysis of maxillofacial granular cell tumor.

OBJECTIVES: To analyze the clinicopathological features of maxillofacial granular cell tumors (GCT) with the aid of immunohistochemical staining. METHODS: Seven cases of maxillofacial GCT were retrospectively collated, and the microscopic morphology of maxillofacial GCT was analyzed. The expression of S-100, neuron-specific enolase (NSE), SOX-10, CD68, actin, desmin, and Ki-67 in GCT was detected by immunohistochemical staining. The cases were observed in the follow-ups after clinical treatment. RESULTS: All seven GCT tumors lacked envelopes and were poorly defined. Microscopically, the sizes of the tumor cells were large and appeared with inconspicuous cell membranes, forming a syncytium-like appearance. The cytoplasm was filled with characteristic eosinophilic granules. The immunohistochemical results showed that six cases were NSE-positive, five cases were S-100-positive, seven cases were CD68-positive, five cases were SOX-10-positive, one case was actin-positive, and seven cases were desmin-negative. The Ki-67 index did not exceed 5% in all cases. In the follow-up sessions, none of the six cases presented a recurrence. CONCLUSIONS: Maxillofacial GCT has a characteristic histological structure. Immunohistochemical S-100, CD68, and other indicators can assist in diagnosis, and the prognosis is good after clinical resection.

Granular cell tumour of the breast: A case report.

INTRODUCTION: Breast granular cell tumour (GCT) is a rare but usually benign lesion. PRESENTATION OF CASE: We report a case of a woman with breast GCT. CONCLUSION: Clinically and radiologically, GCT may mimic breast carcinoma. A conclusive diagnosis is made after a histopathological examination of the lesion. The treatment of choice is surgery.

Clinical outcomes of esophageal granular cell tumors with different endoscopic resection methods.

Esophageal granular cell tumors (GCTs), the second most common subepithelial tumors (SETs) of the esophagus, are potentially malignant with no definite management guidelines available. We retrospectively enrolled 35 patients with endoscopically resected esophageal GCTs between December 2008 and October 2021 and evaluated the clinical outcomes from the various methods performed. Several modified endoscopic mucosal resections (EMRs) were performed for treating esophageal GCTs. Clinical and endoscopic outcomes were evaluated. Mean age of patients was 55.8 ± 8.2, with majority being men (57.1%). Mean tumor size was 7.2 ± 2.6 mm, most (80.0%) were asymptomatic and present in the distal third of the esophagus (77.1%). Endoscopic characteristics predominantly included broad-based (85.7%) and whitish-to-yellowish color changes (97.1%). Endoscopic ultrasound (EUS) of 82.9% of the tumors revealed homogeneous hypoechoic SETs originating from the submucosa. The five endoscopic treatment methods used were: ligation-assisted (77.1%), conventional (8.7%), cap-assisted (5.7%), and underwater (5.7%) EMRs and ESD (2.9%). Mean procedure time was 6.6 ± 2.1 min, and no procedure-associated complications were noted. The en-bloc and complete histologic resection rates were 100% and 94.3%, respectively. No recurrences were noted during follow-up, and no significant differences in the clinical outcomes of the different methods of endoscopic resection were found. Based on tumor characteristics and therapeutic outcomes, modified EMR methods can be effective and safe. However, there were no significant differences in the clinical outcomes of the different methods of endoscopic resection.

Granular cell tumors of the urethra.

Granular cell tumors (GCTs) are a rare type of mesenchymal tumors that are histologically derived by Schwann cells and rise within soft tissues such as skin and mucosal surfaces. Differentiation between benign and malignant GCTs is often difficult and relies on their biological behavior and metastatic potential. While there are no standard guidelines for management, upfront surgical resection, whenever feasible, is key as a definitive measure. Systemic therapy is often limited by poor chemosensitivity of these tumors; however, accumulating knowledge of their underlying genomic landscape has opened some opportunities for targeted approaches, for example, the vascular endothelial growth factor tyrosine kinase inhibitor pazopanib, which is already in clinical use for the treatment of many types of advanced soft tissue sarcomas.

[Pulmonary granular cell tumors: a clinicopathological analysis of five cases].

Objective: To investigate the clinicopathological features of pulmonary granular cell tumors (pGCTs) and to improve the diagnostic accuracy of the tumor. Methods: A total of 5 pGCTs were diagnosed from February 2016 to January 2022 at Shanghai Pulmonary Hospital, Tongji University School of Medicine and Fudan University Shanghai Cancer Center, China. Immunohistochemical staining, and analysis of the clinicopathological characteristics were performed. Results: The average age of the pGCTs patients was 46 years (ranging from 24 to 54 years), with 3 females and 2 males. One case occurred in the bronchus with multiple nodules in the lung, 2 cases occurred in the bronchial opening, and 2 cases were solitary nodules in the lung. The maximum diameter of the tumors ranged from 12 to 15 mm (mean size 14 mm). Microscopically, the tumor showed infiltrative growth and consisted of round, oval or polygonal cells. Abundant eosinophilic cytoplasm was noted, and the nucleoli were prominent. None of the 5 cases showed any mitosis or necrosis. Immunohistochemical and histochemical study showed positive staining for S-100 (5/5), SOX10 (5/5), Vimentin (5/5), TFE3 (4/5), PAS (3/5), and amylase-digested-PAS (3/5), while 4 cases were negative for CD68. TFE3 FISH analyses on 2 cases showed that no signal abnormality was detected in these 2 cases. The average proliferation index of Ki-67 was 2.2% (range 0-5%). There was no recurrence in 4 cases of pGCTs with a follow-up time ranging from 2 months to 60 months. Conclusions: pGCTs are very rare tumors, most likely originating from Schwann cells. Immunohistochemical staining is the conventional diagnostic tool for pGCTs diagnosis. Recognition of this entity is essential for pathologists to avoid misdiagnosis and unnecessary treatments.

Granular cell tumour of the pancreas: a case report and systematic review.

PURPOSE: Granular cell tumours (GCTs) of the pancreas are mostly benign and exceptionally rare, with no unique identifying radiological features. Following a case discussion of a patient with GCT, a comprehensive review of available literature was conducted to identify the common diagnostic features associated with GCT. METHODS: Following a case report identified in our institution, a systematic review was conducted by two authors in accordance with Preferred Reporting Items for Systematic review and Meta-Analysis protocols (PRISMA) guidelines. Databases MEDLINE, EMBASE, Scopus, World of Science, and grey literature were searched on August 2021. Inclusion criteria were histopathology diagnosed granular cell tumour of the pancreas. RESULTS: A 37-year-old male presented with 1 month of abdominal pain and an MRI demonstrating a dilated main pancreatic duct, distal parenchymal atrophy, but no focal lesion. Repeat MRI at 6 months re-demonstrated similar findings and subsequent endoscopic ultrasound was suspicious for main duct IPMN. Following multidisciplinary team discussion, a spleen-preserving distal pancreatectomy was performed. Histopathology demonstrated granular cell tumour with cells diffusely positive for S100 and no malignant transformation. 11 case reports were identified in the literature with diagnosis confirmed on tissue histopathology based on positive immunohistochemical staining for S-100 protein. Eight patients presented with gastrointestinal symptoms with abdominal pain the main presenting complaint (50%). 10 patients underwent CT with portal venous contrast and all underwent endoscopic examination. Imaging findings were similar in five studies for EUS which demonstrated a hypoechoic lesion with homogenous appearance. On non-contrast CT GCT was iso-enhancing, and with portal venous contrast demonstrated hypo-enhancement that gradually enhanced on late phases. Pre-operative diagnosis of pancreatic carcinoma was described in six cases based on imaging and biopsy, resulting in progression to surgical resection. Nine patients were managed surgically and no complications identified on follow-up (6-52 months). CONCLUSION: The currently proposed management pathway includes EUS with biopsy and CT, and surgical resection recommended due to malignancy risk. Improved sample collection with EUS-FNA and microscopic assessment utilising S-100 immunohistochemistry may improve pre-operative diagnosis. Limitations include rare numbers in reported literature and short follow-up not allowing an assessment of GCT's natural history and malignancy risk. Additional cases would expand the current dataset of GCTs of the pancreas, so that surgical resection may be avoided in the future.

Rectal Granular Cell Carcinoma Requiring Abdominoperineal Resection.

BACKGROUND: Granular cell tumors, derived from neural crest cells, are rare tumors infrequently located in the colon or rectum. We will discuss a patient with a rectal granular cell tumor invading the anal sphincters requiring an abdominoperineal resection. METHODS: A 56-year-old male, with anal pain, was found to have a perirectal mass. Pathology from ultrasound-guided transrectal biopsy demonstrated low grade granular cell tumor. The patient underwent a laparoscopic abdominoperineal resection with perineum reconstruction. RESULTS: Pathology demonstrated a granular cell tumor of 4.5 centimeters with tumor invasion of the anal sphincters. Surgical margins were free of neoplasm. DISCUSSION: This is the only documented case of a colorectal granular cell tumor that has required an abdominoperineal resection. On histology, it was considered low grade but its behavior was more consistent with a malignant process. Additional research on malignant granular cell tumors is necessary to help improve treatment options, prevent recurrence, and improve overall survival. His medical course will be followed for disease progression or metastasis.

Granular cell tumour of the cavernous sinus: A rare cause of secondary trigeminal neuralgia.

BACKGROUND: Cavernous sinus tumours comprise 0.1-0.2% of all intracranial tumours, and are most commonly meningiomas or schwannomas. Central nervous system and cranial nerve granular cell tumours (GCTs) are extremely rare. We report the tenth case of a GCT arising from a cranial nerve, and the second case reported in a cavernous sinus location, and review the literature. CLINICAL PRESENTATION: A 67-year-old man presented with right sided trigeminal neuralgia. Imaging findings suggested a trigeminal schwannoma and he was treated with CyberKnife radiosurgery. Over a period of 41 months follow up, there was a progression in both symptoms and imaging findings, requiring debulking surgery. Histopathology identified a GCT. CONCLUSIONS: This is the first case of a cranial nerve GCT treated with stereotactic radiosurgery. Trigeminal nerve GCTs are a rare differential in cases of presumed schwannomas.

Pulmonary granular cell tumors: a clinicopathological analysis of five cases / 中华病理学杂志

Objective: To investigate the clinicopathological features of pulmonary granular cell tumors (pGCTs) and to improve the diagnostic accuracy of the tumor. Methods: A total of 5 pGCTs were diagnosed from February 2016 to January 2022 at Shanghai Pulmonary Hospital, Tongji University School of Medicine and Fudan University Shanghai Cancer Center, China. Immunohistochemical staining, and analysis of the clinicopathological characteristics were performed. Results: The average age of the pGCTs patients was 46 years (ranging from 24 to 54 years), with 3 females and 2 males. One case occurred in the bronchus with multiple nodules in the lung, 2 cases occurred in the bronchial opening, and 2 cases were solitary nodules in the lung. The maximum diameter of the tumors ranged from 12 to 15 mm (mean size 14 mm). Microscopically, the tumor showed infiltrative growth and consisted of round, oval or polygonal cells. Abundant eosinophilic cytoplasm was noted, and the nucleoli were prominent. None of the 5 cases showed any mitosis or necrosis. Immunohistochemical and histochemical study showed positive staining for S-100 (5/5), SOX10 (5/5), Vimentin (5/5), TFE3 (4/5), PAS (3/5), and amylase-digested-PAS (3/5), while 4 cases were negative for CD68. TFE3 FISH analyses on 2 cases showed that no signal abnormality was detected in these 2 cases. The average proliferation index of Ki-67 was 2.2% (range 0-5%). There was no recurrence in 4 cases of pGCTs with a follow-up time ranging from 2 months to 60 months. Conclusions: pGCTs are very rare tumors, most likely originating from Schwann cells. Immunohistochemical staining is the conventional diagnostic tool for pGCTs diagnosis. Recognition of this entity is essential for pathologists to avoid misdiagnosis and unnecessary treatments.

Tumor de células granulares da mama: Relato de caso / Granular cell tumor of the breast: A case report

Os tumores de células granulares (TCG) são tumores raros de tecidos moles que podem ocorrer na mama. Embora quase sempre benignos, eles podem simular um tumor maligno tanto clinicamente quanto em exames de imagem. O objetivo deste trabalho é relatar o caso de uma paciente com TCG da mama. O TCG é uma lesão relativamente rara, em sua maioria benigna, estando geralmente associada a um bom prognóstico. O tratamento cirúrgico continua sendo o padrão pela literatura disponível, podendo ser realizada excisão local ampla com margens cirúrgicas negativas. Considerando o caráter benigno destes tumores, a biópsia do linfonodo sentinela não está indicada de rotina no tratamento cirúrgico. É relatado um certo risco de recorrência, particularmente em caso de margens de ressecção positivas. Em geral são tumores bem circunscritos, mas muitas vezes se apresentam como lesões não encapsuladas, permitindo infiltração no tecido circunjacente, como pele, tecido fibroso, tecido adiposo e músculo peitoral maior. Nenhuma terapia adjuvante específica é sugerida, no entanto, o acompanhamento a longo prazo é fortemente recomendado após a remoção do TCG de mama

Granular cell tumors (GCT) are rare soft tissue tumors that can occur in the breast. Although almost always benign, they can mimic a malignant tumor both clinically and on imaging studies. The aim of this study is to report the case of a patient with GCT of the breast. GCT is a relatively rare lesion, mostly benign, and generally associated with a good prognosis. Surgical treatment remains the standard according to the available literature, and it is recommended a wide local excision with negative surgical margins. Considering the benign nature of these tumors, sentinel lymph node biopsy is not routinely indicated for surgical treatment. A certain risk of recurrence is reported, particularly in case of positive resection margins. They are generally well circumscribed tumors, but often present as non-encapsulated lesions, allowing infiltration into the surrounding tissue, such as skin, fibrous tissue, adipose tissue and pectoralis major muscle. No specific adjuvant therapy is suggested; however, long term follow-up is strongly recommended after breast GCT removal

Granular cell tumour in the ciliary body.

Granular cell tumours (GCTs) are benign tumours that rarely develop in intraocular regions. We report a rare case of intraocular GCT in the ciliary body. A woman in her 20s with a history of bone marrow transplantation for malignant lymphoma in early childhood was referred to our department for bilateral proliferative diabetic retinopathy. A yellowish-white ciliary tumour was observed in the temporal periphery of the patient's left eye during routine ophthalmological examination. As the tumour enlarged, we performed total resection combined with vitrectomy, silicone oil tamponade and cataract surgery. Histopathological examination revealed tumour cells with small, round or oval nuclei with eosinophilic cytoplasm. Positive immunohistochemical staining for S-100 and vimentin led to a diagnosis of ciliary GCT. No retinal detachment, proliferative membrane formation or tumour recurrence was observed 4 years postoperatively. Intraocular GCT should be considered a differential diagnosis of ciliary tumours.

Clinical and pathological analysis of congenital granular cell tumor.

OBJECTIVES: This study aims to explore the clinical and pathological characteristics of congenital granular cell tumors and provide some references for clinical diagnosis, differential diagnosis, and treatment. METHODS: Nine ca-ses of congenital granular cell tumors who visited the Children's Hospital of Zhejiang University School of Medicine from February 2008 to March 2022 were retrospectively analyzed. Herein, its clinical characteristics, pathological characteristics, treatment, and prognosis were summarized and analyzed. RESULTS: We found that nine patients were all female, aged 1­38 days when they saw the doctor. Three of them were attached in maxillary and the other six were attached in mandible. Meanwhile, six tumors were found during the mother's pregnancy at 28-39 weeks and three tumors were found at the baby's birth. One case was excised surgically under local anesthesia, and the other cases were excised surgically under general anesthesia. After 1 month to 12 years of follow-up, patients have no recurrence, however, two cases emerged new teeth from the tumor resection site. Histopathology of all excised lesions was congenital granular cell lesion. CONCLUSIONS: Congenital granular cell tumor is a benign tumor and the prognosis is good. Therefore, surgical resection of the tumor can be done without extensive resection, and it generally does not relapse. Thus, ultrasonography during pregnancy is an important method for the early detection of congenital granular cell epulis.

Rare case of granular cell tumour at 16 years - A case report.

Rationale: Granular cell tumours (GCTs) of the tongue are a rare, soft tissue pathological entity at young ages. This case report aims to present one such case. Patient Concerns: A 16-year-old female patient sought treatment for a small, slow-growing, painless nodule in the dorsum of the tongue since six months. She underwent successful orthodontic treatment in recent past and is in the retention phase. Diagnosis: Excisional biopsy revealed the lesion to be a GCT under histopathology. Treatment: The patient was treated for an abnormal, small, slow-growing, painless nodule in the dorsum of the tongue. Outcomes: The patient had satisfactory esthetics and early diagnosis. The active intervention dispelled the confusion about the role of trauma and orthodontics appliance in the etiology of GCT in this particular case to the patient. Take-away Lessons: GCT can occur at any age. Early diagnosis and corrective surgery would help to avoid late complications.

Granular cell tumor of the pancreas with chronic dilation of the main pancreatic duct.

Although a granular cell tumor (GCT) can originate in various locations, GCT of the pancreas is a rare entity. We herein report a case of GCT of the pancreas with chronic dilation of the main pancreatic duct (MPD). The patient had been referred to our institution for epigastric pain 12 years ago at 50 years old. Multidetector-row computed tomography (MDCT) had shown dilation of the MPD up to 8 mm in diameter from the pancreatic body to the tail. However, she had refused additional workup and been lost to follow-up. She was referred to our institution at 62 years old, as dilation of the MPD had been noted again. Imaging studies showed that the MPD had become dilated up to 33 mm from the pancreatic body to the tail. MDCT showed a hypovascular nodule (6 mm in diameter) in the pancreatic neck, and the nodule was located at the origin of MPD dilation. Pancreatic cancer was considered as a possible differential diagnosis. Surgical treatment was planned for a therapeutic diagnosis, and distal pancreatectomy with splenectomy and lymphadenectomy was performed. The gross findings of the resected specimen revealed a pale-yellow solid tumor measuring 8 mm in diameter at the pancreatic neck. The histopathologic assessment revealed the tumor to be GCT of the pancreas. The characteristics of GCT of the pancreas, such as a tumor causing stenosis and dilation of the MPD frequently and being too small to detect on imaging studies, may lead to a misleading diagnosis of pancreatic cancer.

Granular cell tumor of the appendix: a case report and literature review.

A granular cell tumor (GCT) is an unusual benign soft tissue tumor that can occur at any age and in any part of the body. GCTs are mostly found in the skin and subcutaneous tissues, bronchi, esophagus, breast tissue, and tongue. A GCT originating in the digestive tract, particularly in the appendix, is relatively rare and usually diagnosed as an incidental finding. We herein describe the first case of abdominal distension and occasional pain secondary to a GCT of the appendix in our hospital. The findings from this case suggest that a GCT of the appendix is a rare entity for which surgical resection is an efficient therapy.