Gcts are thought to originate from nerve cells, particularly schwann cells. Granular cell astrocytoma gca, which has also been called intracerebral granular cell tumor or astrocytoma with granular cell differentiation, is a rare type of infiltrative malignant brain tumor. A clinical and pathologic study of cases the annals of thoracic surgery. Fineneedle aspiration cytology of granular cell tumor. A distinctive entity further delineated in a clinicopathological study of 11 cases. Granular cell tumor of skin diagnosed on fine needle aspiration cytology. Less than 1 in 50 gcts would have tendency to become malignant.
This is an example of multicentric granular cell tumour case number 10. The tumour usually occurs in adults but a few cases have been reported in children. The tumour cells are large in size, with small, uniform, eosinophilic granules filling the cytoplasm, and small, roundtooval nuclei figures. It was first described in the bronchus by kramer in 1939.
Histopathology tonguesquamous cell carcinoma duration. Malignant granular cell tumor can occur at any age, but is more commonly seen in ages between 3050 years. On the left side a, the tumour is poorly circumscribed solid and pale yellow, and shows infiltrative growth margin paraspinal tumour. Granular cell tumour, previously known as granular cell myoblastoma, is an unusual tumour of schwann cell origin. They are most commonly observed in the oral cavity, skin and subcutaneous tissue, breast, and. It was believed to originate from muscles and so were named myoblastenmyoma.
S100, nse were negative, suggesting that gcot is a distinct entity compared with granular cell tumour. Lopezarranz departmentof oral and maxillofacialsurgery, central hospital, oviedo. Granular cell tumor gct is an uncommon tumor with a predilection for the skin, soft tissue, and mucosa of the upper aerodigestive tract, particularly the tongue and vocal cords. Most are benign and reportedly malignant cases are rare, occurring in only 1% or 2% of cases. American journal of clinical pathology, volume 148, issue 2, august 2017, pages 161 166. Malignant granular cell tumor is a very rare variant of the granular cell tumor. Metastasizing malignant granular cell tumor abrikossoff. Departmentof morphologyand cell biology, medical school, university of oviedo, spain summary. These tumors are of neural origin, and they are called granular cell tumors because of their appearance under the microscope. Granular cell tumors gcts of the infundibulum are rare in practice and literature, resulting in a lack of evidencebased standard of care. It most commonly occur in tongue but can occur at any other sites presenting as an asymptomatic, solitary, flesh coloured, firm or hard nodule in middle aged persons 1. Immunohistochemical investigation confirmed the diagnosis.
As granular cell ameloblastoma shows positive calretinin staining and ck staining in both peripheral and central cells. A battery of immunohistochemical stains is needed to make a specific diagnosis. We identified eleven cases in ten patients treated surgically and. Granular cell tumors gcts are uncommon, benign soft tissue neoplasms originating in the schwann cells of the nerve sheath. Less common sites include deep soft tissues and visceral organs. Granular cell tumors may produce skin or deep fascial fixation and hence may be clinically and grossly indistinguishable from scirrhous breast carcinoma. Morphometrical differences between pseudoepitheliomatous hyperplasia in granular cell tumours and squamous cell carcinomas. They often present as asymptomatic, slowgrowing, benign, solitary lesions but may be multifocal. The tumour cells are large in size, with small, uniform. Granular cell tumors gcts are extremely rare lesions of the urinary bladder with only nine cases being reported in world literature of which one was malignant. Their name derives from the fact that an accumulation of cytoplasmic lysosomes imparts the tumor with a granular appearance. They present as small painless nodules in the dermis, subcutis, or mucosal surfaces.
Tumors are also called neoplasms, which means that they are composed of new and actively growing tissue. Intraoral sites tongue, oral mucosa, hard palate are frequently involved. The granular cell tumors gcts are rare benign soft tissue neoplasm of nerve sheath origin. Granular cell tumour of the colon with extensive sclerosis. Typical and atypical granular cell tumors of soft tissue american. It is also known as abrikossoffs tumor, granular cell myoblastoma, granular cell nerve sheath tumor, and granular cell schwannoma. Granular cell tumors gcts, abrikossoff tumors are rare benign soft tissue tumors that were first described in 1962. Granular cell tumor genetic and rare diseases information. However, there remains a subcategory of granular cell tumour, first described by le boit as primitive polypoid granular cell tumour, that shows no obvious line of differentiation.
Aug 23, 2018 granular cell tumors gcts of the infundibulum are rare in practice and literature, resulting in a lack of evidencebased standard of care. Gct is an uncommon tumour of schwann cell origin with an incidence of 0. Dec 29, 2018 granular cell tumors may produce skin or deep fascial fixation and hence may be clinically and grossly indistinguishable from scirrhous breast carcinoma. Granular cell tumor histology, causes, symptoms, diagnosis. However, determination of malignant potential and appropriate management are as yet unresolved issues. The first reported case of granular cell tumor involving the tongue was in 1926 by. A granular cell tumour grcts is a rare, benign, soft tissue tumour that likely arises from schwann cells. Cutaneous and soft tissue granular cell tumour is a well. The panmacrophage marker, cd68 kp1 reacts strongly with granular cell tumour. The tongue and skin are the most common sites of involvement. Multiple morphologically distinct cutaneous granular cell. Malignant granular cell tumor mgct is a rare highgrade mesenchymal tumor of schwann cell origin.
Histopathology revealed focal ulceration with underlying stroma demonstrating large sheets of closely packed, polygonal cells with round, regular nuclei and inconspicuous nucleoli, and abundant granular cytoplasm figure 5, consistent with a diagnosis of epulis, or granular cell tumour gct. Granular cell tumor is a tumor that can develop on any skin or mucosal surface, but occurs on the tongue 40% of the time. Granular cell tumours gcts are uncommon in the gastrointestinal tract, particularly in the colorectum. The diagnosis of granular cell tumour is made by skin biopsy. Tumour size more than 5 cms, presence of vascular invasion, necrosis and rapid growth. Granular cell tumors diagnostic difficulties, as its histopathological features can be confused with a welldistinguished oral squamous cell. Granular cell tumor gct is a benign lesion that occurs at various sites. Granular cell tumour is a rare, benign tumour that may arise in the soft tissues of various locations. Histopathological features of granular cell tumor medcraveonline. A general medical history and examination to exclude any potentially associated conditions are useful. Multiple cutaneous gcts in a single patient have been reported, with the lesions being described as subcutaneous papules, nodules, or verrucous nodules. Fewer than 200 cases of esophageal granular cell tumors gct have been reported. Adverse prognostic factors include local recurrence, metastasis, larger tumor. Introduction granular cell tumours grcts are uncommon soft tissue tumours that are usually benign approximately 0.
Granular cell tumors gct, previously referred to as granular cell myoblastoma are rare mesenchymal tumors, comprising about 0. Dermal nonneural granular cell tumour socalled primitive polypoid granular cell tumour. Adverse prognostic factors include local recurrence, metastasis, larger tumor size, older patient age, histologic classification as malignant, presence of necrosis, increased mitotic activity, spindling of tumor cells. These slowgrowing, benign tumors of neural origin occur anywhere in the body are generally asymptomatic, usually solitary, although rarely multifocal. Central cells with granular cytoplasm and eccentrical. Granular cell tumors gcts are soft tissue tumors that can occur anywhere in the body. Granular cell tumours are rare mesenchymal soft tissue tumours that arise throughout the body and are believed to be of neural origin. Generally believed to be of neural origin based on histochemical, immunohistochemical, and ultrastructural studies.
Dec 29, 2018 granular cell variants of basal cell carcinoma, melanoma, leiomyoma, leiomyosarcoma, dermatofibrosarcoma, angiosarcoma, fibrous histiocytoma, and ameloblastoma can sometimes be indistinguishable from granular cell tumors if examined with routine light microscopy. Although it was initially classified as a myoblastoma, recent studies agree that it is more likely to be neural in origin. Histopathology reveals characteristic granules within the cytoplasm of large tumour cells. Pathology of granular cell tumour dr sampurna roy md. The histopathological examination of the excised specimen revealed no. We report a case of a 73 yearold man with an incidentally. Discussion congenital gingival granular cell tumours have usually been. Most authors favor a schwann cell origin, based on histochemical, immunohistochemical, and ultrastructural findings and on occurrence of typical lesions, within nerves. Granular cell tumour is also known as abrikossoff tumour. Diagnostics and therapeutic aspects exemplified by a. It is thought to have a neural origin, probably derived from schwann cells. Granular cell tumours grcts are uncommon soft tissue tumours that are. We report on our findings in six patients who developed granular cell tumor in the mammary gland, esophagus.
Herein, we report a series of 30 colorectal gcts and discuss the properties of colorectal gcts based on histopathological and immunohistochemical studies. A malignant granular cell tumor excised with mohs micrographic surgery. Granular cell tumor gct is a rare benign neoplasm of the skin that accounts for 0. Later, gca was found to be a highly aggressive neoplasm, in dramatic contrast to the benign. Granular cell variants of basal cell carcinoma, melanoma, leiomyoma, leiomyosarcoma, dermatofibrosarcoma, angiosarcoma, fibrous histiocytoma, and ameloblastoma can sometimes be indistinguishable from granular cell tumors if examined with routine light microscopy. All these features were suggestive of granular cell tumour. They were first reported in 1920 by abrikossoff in a case series composed of benign tumors that were removed from the tongue. We report a 50yearold female who had mgct arising in the anterior abdominal wall and developed massive metastatic. A granular cell tumor grct of the breast is an uncommon, usually benign tumor which is possibly of neural origin.
Treatment of congenital epulis granular cell tumour with. Epithelial islands surrounded by connective tissue. Bishop, in head and neck pathology third edition, 2019. Histology confirmed that excision margins were clear of the tumour. Granular cell tumor of the lung is a rare neoplasm comprising 610% of all granular cell tumor. May 14, 2007 histopathology skin granular cell tumor. A systematic literature search was conducted according to prisma. In granular cell tumour, sections show broad fascicles of tumour cells arranged in nests or sheets infiltrating the dermis and dermal structures.
Pathology of granular cell tumour of bronchus dr sampurna. Granular cell tumor grct also known as schwannoma or abrikossoff tumor, is a rare soft tissue tumor derived from schwann cells. Three months postoperatively the child is thriving with no evidence of recurrence. Granular cell tumors gcts affect females more often than males. Clinical and histopathological features of orbital granular cell tumor.
Granular cell differentiation wiley online library. Some evidence suggests that it originates from schwann cells, with positivity for s100 protein. Granular cell tumor is a soft tissue neoplasm that originates in the nervous system and arises at virtually any body site, but is mainly found in the skin, oral cavity or digestive tract. To the best of our knowledge, only two genuine cases of granular cell tumour have been described in the thyroid gland. Granular cell tumour gct is a rare, usually benign, mesenchymal lesion that can occur in nearly any organ, but has a proclivity for the oral cavity, skin and subcutaneous tissue.
They are thought to arise from the cells that surround and insulate the nerve cells in our body schwann cells. Granular cell tumour of the thyroid gland, histopathology. Granular cell tumors gcts are rare and benign tumors that can occur at any anatomical site. On the right side b the tumour shows better defined pushing margin supraclavicular tumour. Granular cell tumour of the breast diagnostic radiology case study introduction granular cell tumor gct is a rare neoplasm that may occur in any anatomical part of the body, most commonly within the oral cavity, the tongue.
It has poor prognosis with 39% mortality rate in 3year interval. Granular cell tumor gct is a rare soft tissue neoplasm that can virtually affect any site of the body. The granular cell tumour is a rare histomorphologically distinctive neoplasm found at many sites. Oct 25, 2016 granular cell tumors gcts are soft tissue tumors that can occur anywhere in the body. They commonly arise on lower extremity, nuchal region, chest wall, gastrointestinal tract. A systematic literature search was conducted according to. Granular cell tumors are rare neoplasms of neural origin. Introduction granular cell tumors, also called abrikossoff tumors, are benign, slowly growing neoplasms. Granular cell odontogenic tumour a histopathological rarity. Malignant granular cell tumors behave similarly to malignant peripheral nerve sheath tumors and have a 50% rate of metastasis. They may occur anywhere in the body, but head and neck accounts to 45% 65% of these cases 1. Histopathology images are shown in figures 1 and 2. They occasionally arise in the gastrointestinal tract git where the oesophagus is most commonly involved.
The nodule appeared very close to the posterior wall of the specimen. Epidemiology they tend to occur at a younger age than other types of breast cancer 4. It is important that clinicians are aware of their existence. At the microscopic level, approximately 50% of granular cell tumors have a diffuse infiltrative pattern and induce a stromal response similar to that of scirrhous breast carcinoma. Conclusions granular cell tumor gct is a benign soft tissue neoplasm. The breast accounts of 58% of all granular cell tumors 2. Multiple cutaneous granular cell tumors and neurofibromatosis in childhood. Grcts can occur in a variety of visceral and cutaneous sites. Granular cell tumor is a tumor that can develop on any skin or mucosal surface, but occurs on the tongue 40% of the time it is also known as abrikossoffs tumor, granular cell myoblastoma, granular cell nerve sheath tumor, and granular cell schwannoma. It was first described by markesbery et al1 in 1973 as granular cell tumor in the central nervous system. Granular cell tumor gct is a benign neural tumor containing polygonal cells with abundant granular eosinophilic cytoplasm. Granular cell tumor definition of granular cell tumor by. Granular cell tumour of the breast diagnostic radiology.
Granular cell tumours gct of the soft tissues are rare benign tumours but some time may be difficult to distinguish from malignant neoplasms. Jun 30, 2016 granular cell tumour gct is a rare, usually benign, mesenchymal lesion that can occur in nearly any organ, but has a proclivity for the oral cavity, skin and subcutaneous tissue. Granular cell tumor an overview sciencedirect topics. Pdf granular cell tumor gct is uncommon, mostly benign neoplasm that can affect any organ. The tumour cells show positivity with s100 protein, neuronspecific enolase, vimentin, pgp9. Histopathology confirmed the diagnosis of a completely excised congenital gingival granular cell tumour figure 2. Initially, gcts were believed to occur in skeletal muscles only. In contrast, 11 of 28 patients 39% with malignant granular cell tumor with followup information died of disease at a median interval of 3 years.