Granular cell tumors gcts are soft tissue tumors that can occur anywhere in the body. Granular cell tumors are rare neoplasms of neural origin. 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 gct is a benign neural tumor containing polygonal cells with abundant granular eosinophilic cytoplasm. Typical and atypical granular cell tumors of soft tissue american.
The first reported case of granular cell tumor involving the tongue was in 1926 by. Epidemiology they tend to occur at a younger age than other types of breast cancer 4. Malignant granular cell tumor is a very rare variant of the granular cell tumor. 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. Less common sites include deep soft tissues and visceral organs. Multiple cutaneous granular cell tumors and neurofibromatosis in childhood. 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. They may occur anywhere in the body, but head and neck accounts to 45% 65% of these cases 1. The tumour cells show positivity with s100 protein, neuronspecific enolase, vimentin, pgp9.
On the right side b the tumour shows better defined pushing margin supraclavicular tumour. Granular cell tumor gct is a rare soft tissue neoplasm that can virtually affect any site of the body. Granular cell tumors gcts are rare and benign tumors that can occur at any anatomical site. Granular cell tumors gct are uncommon benign soft tissue neo plasms that have a predilection for the head and neck region. They occasionally arise in the gastrointestinal tract git where the oesophagus is most commonly involved. Initially, gcts were believed to occur in skeletal muscles only. Malignant granular cell tumor can occur at any age, but is more commonly seen in ages between 3050 years. Granular cell tumour on vaccination scar in a young girl. 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. They are most commonly observed in the oral cavity, skin and subcutaneous tissue, breast, and. Lopezarranz departmentof oral and maxillofacialsurgery, central hospital, oviedo. We report a case of a 73 yearold man with an incidentally. Majority are benign with excellent outcome after surgical resection some propensity for local recurrence if incompletely excised j surg oncol 1980. The tumor cells of granular cell tumor are large polygonal cells with abundant, pale amphophilic to eosinophilic, granular cytoplasm and small round nuclei fig.
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. Granular cell tumour of the breast diagnostic radiology. Three months postoperatively the child is thriving with no evidence of recurrence. 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. 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. Granular cell tumour of the thyroid gland, histopathology. Less than 1 in 50 gcts would have tendency to become malignant. Conclusions granular cell tumor gct is a benign soft tissue neoplasm. The histopathological examination of the excised specimen revealed no. Granular cell tumour is also known as abrikossoff tumour. A battery of immunohistochemical stains is needed to make a specific diagnosis. All these features were suggestive of granular cell tumour. Immunohistoichemistry showed s100 to be diffusely positive, while nse faintly positive.
Bishop, in head and neck pathology third edition, 2019. They commonly arise on lower extremity, nuchal region, chest wall, gastrointestinal tract. Granular cell tumor gct is a rare benign neoplasm of the skin that accounts for 0. We report on our findings in six patients who developed granular cell tumor in the mammary gland, esophagus. 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. Granular cell tumor of the lung is a rare neoplasm comprising 610% of all granular cell tumor. Intraoral sites tongue, oral mucosa, hard palate are frequently involved. Clinical and histopathological features of orbital granular cell tumor.
Later, gca was found to be a highly aggressive neoplasm, in dramatic contrast to the benign. Central cells with granular cytoplasm and eccentrical. Pathology of granular cell tumour dr sampurna roy md. Tumour size more than 5 cms, presence of vascular invasion, necrosis and rapid growth. They present as small painless nodules in the dermis, subcutis, or mucosal surfaces. Some evidence suggests that it originates from schwann cells, with positivity for s100 protein. Gct is an uncommon tumour of schwann cell origin with an incidence of 0. Adverse prognostic factors include local recurrence, metastasis, larger tumor.
Histopathology reveals characteristic granules within the cytoplasm of large tumour cells. However, determination of malignant potential and appropriate management are as yet unresolved issues. Granular cell tumours gcts are uncommon in the gastrointestinal tract, particularly in the colorectum. 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.
Histology confirmed that excision margins were clear of the tumour. American journal of clinical pathology, volume 148, issue 2, august 2017, pages 161 166. The diagnosis of granular cell tumour is made by skin biopsy. Based on histopathological findings the final diagnosis of gct was made. Malignant granular cell tumors behave similarly to malignant peripheral nerve sheath tumors and have a 50% rate of metastasis. A systematic literature search was conducted according to prisma.
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. It is thought to have a neural origin, probably derived from schwann cells. Granular cell tumor histology, causes, symptoms, diagnosis. Histopathological features of granular cell tumor medcraveonline. Granular cell tumors gcts typically are benign solitary tumors derived from schwann cells. A distinctive entity further delineated in a clinicopathological study of 11 cases. The tumour usually occurs in adults but a few cases have been reported in children. Granular cell odontogenic tumour a histopathological rarity. A granular cell tumor grct of the breast is an uncommon, usually benign tumor which is possibly of neural origin. It was first described by markesbery et al1 in 1973 as granular cell tumor in the central nervous system.
The granular cell tumors gcts are rare benign soft tissue neoplasm of nerve sheath origin. The tumour cells are large in size, with small, uniform. We identified eleven cases in ten patients treated surgically and. The panmacrophage marker, cd68 kp1 reacts strongly with granular cell tumour. A general medical history and examination to exclude any potentially associated conditions are useful. Granular cell tumor grct also known as schwannoma or abrikossoff tumor, is a rare soft tissue tumor derived from schwann cells. Metastasizing malignant granular cell tumor abrikossoff. These tumors are of neural origin, and they are called granular cell tumors because of their appearance under the microscope. Fewer than 200 cases of esophageal granular cell tumors gct have been reported. 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. Morphometrical differences between pseudoepitheliomatous hyperplasia in granular cell tumours and squamous cell carcinomas. It was believed to originate from muscles and so were named myoblastenmyoma. Treatment of congenital epulis granular cell tumour with. Histopathology images are shown in figures 1 and 2.
On the left side a, the tumour is poorly circumscribed solid and pale yellow, and shows infiltrative growth margin paraspinal tumour. Granular cell tumours grcts are uncommon soft tissue tumours that are. Granular cell tumour, previously known as granular cell myoblastoma, is an unusual tumour of schwann cell origin. Malignant granular cell tumor mgct is a rare highgrade mesenchymal tumor of schwann cell origin. It has poor prognosis with 39% mortality rate in 3year interval. They were first reported in 1920 by abrikossoff in a case series composed of benign tumors that were removed from the tongue. 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 tumor definition of granular cell tumor by.
The tumour cells are large in size, with small, uniform, eosinophilic granules filling the cytoplasm, and small, roundtooval nuclei figures. Immunohistochemical investigation confirmed the diagnosis. Fineneedle aspiration cytology of granular cell tumor. The tongue and skin are the most common sites of involvement. Pathology of granular cell tumour of bronchus dr sampurna. Diagnostics and therapeutic aspects exemplified by a. Granular cell tumors gcts, abrikossoff tumors are rare benign soft tissue tumors that were first described in 1962. Granular cell tumors gcts of the infundibulum are rare in practice and literature, resulting in a lack of evidencebased standard of care. Granular cell tumours gct of the soft tissues are rare benign tumours but some time may be difficult to distinguish from malignant neoplasms. A systematic literature search was conducted according to. A malignant granular cell tumor excised with mohs micrographic surgery. Granular cell tumors gcts are uncommon, benign soft tissue neoplasms originating in the schwann cells of the nerve sheath. 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.
Most are benign and reportedly malignant cases are rare, occurring in only 1% or 2% of cases. Primitive polypoid granular cell tumor and other cutaneous granular cell neoplasms of apparent nonneural origin. Granular cell tumour of the colon with extensive sclerosis. Histopathology confirmed the diagnosis of a completely excised congenital gingival granular cell tumour figure 2. 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. Multiple cutaneous gcts in a single patient have been reported, with the lesions being described as subcutaneous papules, nodules, or verrucous nodules. Dermal nonneural granular cell tumour socalled primitive polypoid granular cell tumour.
These slowgrowing, benign tumors of neural origin occur anywhere in the body are generally asymptomatic, usually solitary, although rarely multifocal. They are thought to arise from the cells that surround and insulate the nerve cells in our body schwann cells. To the best of our knowledge, only two genuine cases of granular cell tumour have been described in the thyroid gland. The breast accounts of 58% of all granular cell tumors 2. Pdf granular cell tumor gct is uncommon, mostly benign neoplasm that can affect any organ. The nodule appeared very close to the posterior wall of the specimen. A granular cell tumour grcts is a rare, benign, soft tissue tumour that likely arises from schwann cells. Granular cell differentiation wiley online library. Most granular cell tumors are benign noncancerous, although some may be locally aggressive. 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. 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. It was first described in the bronchus by kramer in 1939. Histopathology tonguesquamous cell carcinoma duration. Granular cell tumor is poorly circumscribed and unencapsulated and typically grows as sheets or vague fascicles fig.
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. Herein, we report a series of 30 colorectal gcts and discuss the properties of colorectal gcts based on histopathological and immunohistochemical studies. In granular cell tumour, sections show broad fascicles of tumour cells arranged in nests or sheets infiltrating the dermis and dermal structures. Introduction granular cell tumours grcts are uncommon soft tissue tumours that are usually benign approximately 0.
Granular cell tumor an overview sciencedirect topics. Discussion congenital gingival granular cell tumours have usually been. We report a 50yearold female who had mgct arising in the anterior abdominal wall and developed massive metastatic. Granular cell tumors gct, previously referred to as granular cell myoblastoma are rare mesenchymal tumors, comprising about 0. S100, nse were negative, suggesting that gcot is a distinct entity compared with granular cell tumour. Epithelial islands surrounded by connective tissue. Granular cell tumor gct is a benign lesion that occurs at various sites. Tumors are also called neoplasms, which means that they are composed of new and actively growing tissue. As granular cell ameloblastoma shows positive calretinin staining and ck staining in both peripheral and central cells. This is an example of multicentric granular cell tumour case number 10. Their name derives from the fact that an accumulation of cytoplasmic lysosomes imparts the tumor with a granular appearance. Generally believed to be of neural origin based on histochemical, immunohistochemical, and ultrastructural studies. It is also known as abrikossoffs tumor, granular cell myoblastoma, granular cell nerve sheath tumor, and granular cell schwannoma.
Granular cell tumor genetic and rare diseases information. May 14, 2007 histopathology skin granular cell tumor. Departmentof morphologyand cell biology, medical school, university of oviedo, spain summary. It is important that clinicians are aware of their existence. Granular cell tumors diagnostic difficulties, as its histopathological features can be confused with a welldistinguished oral squamous cell.
Cutaneous and soft tissue granular cell tumour is a well. 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. They often present as asymptomatic, slowgrowing, benign, solitary lesions but may be multifocal. Gcts are thought to originate from nerve cells, particularly schwann cells. Granular cell tumor of skin diagnosed on fine needle aspiration cytology. Multiple morphologically distinct cutaneous granular cell.
Granular cell tumour is a rare, benign tumour that may arise in the soft tissues of various locations. The granular cell tumour is a rare histomorphologically distinctive neoplasm found at many sites. Most authors favor a schwann cell origin, based on histochemical, immunohistochemical, and ultrastructural findings and on occurrence of typical lesions, within nerves. Granular cell tumours are rare mesenchymal soft tissue tumours that arise throughout the body and are believed to be of neural origin. 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 tumors gcts affect females more often than males. Although it was initially classified as a myoblastoma, recent studies agree that it is more likely to be neural in origin. 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.
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