Granular cell tumor histology, causes, symptoms, diagnosis. The patient was well with no surgical complications or tumor recurrences after one year follow up. Granular cell tumors have a variable clinical appearance and may mimic malignant neoplasms eg, squamous cell carcinoma as well as infectious diseases eg, condyloma, syphilis. The majority of gct are benign, although some display malignant behavior. Granular cell tumor gct is a rare tumor of nerve sheath origin with a. Metastasizing malignant granular cell tumor abrikossoff tumor of.
Malignant granular cell tumor can occur at any age, but is more commonly seen in ages between 3050 years. Histopathology images of tumor, granular cell, breast by. May 14, 2007 histopathology skin granular cell tumor. They are thought to arise from the cells that surround and insulate the nerve cells in our body schwann cells. Sep 10, 2019 granular cell tumors are thought to start in early forms of nerve cells. Cgcl is a rare lesion of the newborn with an incidence of just 0. Granular cell tumor an overview sciencedirect topics. Malignant granular cell tumor is a very rare variant of the granular cell tumor. Our findings suggest that soft tissue granular cell tumors may be slightly more aggressive than their dermal or organconfined counterparts. Granular cell tumor grct also known as schwannoma or abrikossoff tumor, is a rare soft tissue tumor derived from schwann cells. Granular cell tumour of the thyroid gland, histopathology.
Granular cell tumors gcts were firstly described by weber in 1854. Primitive nonneural granular cell tumor socalled atypical polypoid granular cell tumor. Multiple cutaneous gcts in a single patient have been reported, with the lesions being described as subcutaneous papules, nodules, or verrucous nodules. The tumor cells of granular cell tumor are large polygonal cells with abundant, pale amphophilic to eosinophilic, granular cytoplasm and small round nuclei fig. Gcts are thought to originate from nerve cells, particularly schwann cells. Granular cell tumors are usually presents as a single nodule under the skin. Clinical and histopathological features of orbital. The gold standard for diagnosis of granular cell tumor is histopathology. A systematic literature search was conducted according to prisma. What is the anatomic pathology of granular cell tumors gcts. Granular cell tumors are thought to start in early forms of nerve cells. They are most commonly observed in the oral cavity, skin and subcutaneous tissue, breast, and. Granular cell tumors gcts typically are benign solitary tumors derived from schwann cells. Clinical and imaging features of granular cell tumor of.
Granular cell tumours can occur in anyone however, they tend to be more common in females and darkskinned people. The tumor cells were positive for s100 protein fig. Fifteen cases of granular cell tumour of superficial soft tissues or tongue were immunohistochemically evaluated for different types of intermediate filament proteins and for laminin, a glycoprotein of. Granular cell tumor gct is a rare benign neoplasm of the skin that accounts for 0. The patient consented to the publication of his case.
Granular cell odontogenic tumour a histopathological rarity. The granular cell tumors are uncommon, mostly benign neoplasm of neural differentiation that can occur at any site of body. Granular cell tumor definition of granular cell tumor by. Mammogram and ultrasound in a patient with granular cell tumors.
A systematic literature search was conducted according to. P rimitive nonneural granular cell tumors of skin primitive nonneural granular cell tumor of the skin was first described by leboit et al in 1991 as primitive polypoid granular cell tumor. Granular cell tumors are not associated with lymphadenopathy and the overlying epithelium is typically hyperplastic, giving a pseudoepitheliomatous appearance. Granular cell tumors are rare tumors derived from the nerve sheath, are mostly benign tumors, and the incidence of malignancy is 2%. Solitary, multiple, benign, atypical, or malignant. 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. Recurrence risk and margin status in granular cell tumors of the breast a clinicopathologic study of patients. Report of 2 cases with immunohistochemical and ultrastructural correlation. It was first described by markesbery et al1 in 1973 as granular cell tumor in the central nervous system. Histopathological features of granular cell tumor medcrave. Granular cell tumor is not sensitive to radiotherapy and chemotherapy, and needs to. Intraoral sites tongue, oral mucosa, hard palate are frequently involved. Granular cell tumour is also known as abrikossoff tumour.
The diagnosis of the gcts requires histopathological examination of the excised lesion. 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. We report on our findings in six patients who developed granular cell tumor in the mammary. Typical and atypical granular cell tumors of soft tissue. We report on our findings in six patients who developed granular cell tumor in the mammary gland, esophagus. Morphology of the tumor on hematoxylin and eosin h. Granular cell tumor of skin diagnosed on fine needle aspiration cytology. Clinical and histopathological features of orbital granular cell tumor.
May mimic welldifferentiated squamous cell carcinoma histopathologically. The histopathology hpe report was granular cell tumor, inadequate excision 4 figures 15 and tables 1 and 2. Granular cell tumors gct are uncommon benign soft tissue neo plasms that have a predilection for the head and neck region. Congenital granular cell lesion cgcl is a rare benign soft tissue tumor of the newborn. Granular cell tumour of the breast diagnostic radiology. These tumors are of neural origin, and they are called granular cell tumors because of their appearance under the microscope.
Recurrence risk and margin status in granular cell tumors. Histopathological features of granular cell tumor author. In granular cell tumour, sections show broad fascicles of tumour cells arranged in nests or sheets infiltrating the dermis and dermal structures. A predominantly dermal based tumor composed of cells with abundant eosinophilic to basophilic granular cytoplasm. Tumors are also called neoplasms, which means that they are composed of new and actively growing tissue. F ernandes 1, r ubens b elfort n eto 1,2, a lexandre n akao o dashiro 1,3, p atricia r usa p ereira 1,2,m iguel 1,2n. Discussion 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. A rare granular cell tumor with a center ulcer of the rectum. Typical and atypical granular cell tumors of soft tissue a. Compared with granular cell tumors overall, the soft tissue subset shows a larger average size and higher propensity for incomplete resections, with atypical features being relatively common. Granular cell tumors gcts of the infundibulum are rare in practice and literature, resulting in a lack of evidencebased standard of care. Multiple morphologically distinct cutaneous granular cell.
Histopathologic features of a rectal granular cell tumor. 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. There are very few cases of granular cell tumor of the urinary bladder reported in the literature 23 4 5 granular cell tumor was described as myoblastoma on the assumption that it was of. Granular cell tumors gcts typically present as solitary lesions. Granular cell tumor gct is a rare soft tissue neoplasm that can virtually affect any site of the body. The tumor was diagnosed as a benign granular cell tumor of the gallbladder fig. 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. Multiple granular cell tumours may occur in up to 25% of cases and it is in these patients that associations are occasionally made with other conditions. Boyce and beadles, in 1893, were considered the first to recognize gct of the neurohypophysis as a distinct entity, and the tumor was described in more. They most commonly present in darkskinned, middleaged women but have been reported in all age groups and in both sexes. It has poor prognosis with 39% mortality rate in 3year interval. Malignant granular cell tumor mgct is a rare highgrade. Majority are benign with excellent outcome after surgical resection some propensity for local recurrence if incompletely excised j surg oncol 1980.
The clinical evolution and biology of granular cell tumors gct are poorly understood and treatment remains an issue of discussion. Granular cell tumours gcts are uncommon in the gastrointestinal tract, particularly in the colorectum. Recurrence risk and margin status in granular cell tumors of the. Less than 1 in 50 gcts would have tendency to become malignant. Pathology of granular cell tumour dr sampurna roy md. A granular cell tumor of the breast can most often be felt as a firm lump that you can move. Granular cell tumor presenting in the scrotum of a pediatric patient. A case report of a rare intramuscular granular cell tumor diagnostic.
About 1 in 10 individuals with gct may have multiple tumors on the body. We report a 50yearold female who had mgct arising in the anterior abdominal wall and developed massive metastatic. The granular cell tumors gcts are rare benign soft tissue neoplasm of nerve sheath origin. Metastasizing malignant granular cell tumor abrikossoff. Adverse prognostic factors include local recurrence, metastasis, larger tumor. A 3mm punch biopsy was performed, and the histopathological examination of he. The distinction between benign, atypical, and malignant gct is controversial due to morphological and immunohistochemical overlap and lack of consistent histological and phenotypic criteria. Granular cell tumor is important because of its ability to grossly and microscopically simulate the appearance of invasive carcinoma.
There is a welldescribed phenomenon called pseudoepitheliomatous hyperplasia. Desmoplastic primitive nonneural granular cell tumor of the skin. Granular cell tumors gcts are soft tissue tumors that can occur anywhere in the body. Recurrence risk and margin status in granular cell tumors of. Granular cell tumour, previously known as granular cell myoblastoma, is an unusual tumour of schwann cell origin. There were 11 female and eight male esophageal gct patients with a median age. Granular cell tumor johns hopkins surgical pathology. Less common sites include deep soft tissues and visceral organs.
Granular cell tumor, first described in 1856, has a long history of names eg, myoblastic myoma implying putative pathogenesis. Also known as abrikossoffs tumor and granular cell. The tongue and skin are the most common sites of involvement. Granular cell tumor has not been described in rabbits before and has not been described in the testes of animals or postnatal humans. It is thought to have a neural origin, probably derived from schwann cells. Esophageal granular cell tumors gcts are rare and often misdiagnosed. On section, they are firm, homogeneous, and white or grayish yellow. Keywords granular cell tumor, gastrointestinal tumors, biliary tract ann gastroenterol 2018. Granular cell tumors gcts are rare and benign tumors that can occur at any anatomical site. Most of them are benign and reportedly malignant cases are rare, occurring in only 1% or 2% of cases 47. A malignant lesion can be suspected on clinical presentation and confirmed via histopathological examination using the fanburgsmith criteria. Granular cell tumor gct is a benign neural tumor containing polygonal cells with abundant granular eosinophilic cytoplasm. Granular cell tumor of the esophagus presenting as a duplication cyst.
The distinction between benign, atypical, and malignant gct is controversial due to morphological and immunohistochemical overlap and lack of consistent histological and phenotypic. Pdf granular cell tumor gct is uncommon, mostly benign neoplasm that can affect any organ. Granular cell tumors gcts appearing in the larynx are reported to be as rare as 3% to 10% of all cases. 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. They commonly arise on lower extremity, nuchal region, chest wall, gastrointestinal tract. Granular cell tumor genetic and rare diseases information. Most granular cell tumors are benign noncancerous, although some may be locally aggressive. Most are benign and reportedly malignant cases are rare, occurring in only 1% or 2% of cases. 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. Abundant cytoplasmic lysosomes are responsible for the tumor cells granular appearance.
Herein, we report a series of 30 colorectal gcts and discuss the properties of colorectal gcts based on histopathological and immunohistochemical studies. We present a histopathology case report of a gct tumor in 26 year old female. Their name derives from the fact that an accumulation of cytoplasmic lysosomes imparts the tumor with a granular appearance. Sep 18, 2018 what are the signs and symptoms of granular cell tumor.
Clinical and imaging features of granular cell tumor of the. Apr 09, 2008 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 battery of immunohistochemical stains is needed to make a specific diagnosis. Granular cell differentiation wiley online library. Discussion 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.
Histogenesis of granular cell tumouran immunohistochemical. Polypoid s100negative granular cell tumor of the oral cavity. Feb 01, 2004 read granular cell tumour of the thyroid gland, histopathology on deepdyve, the largest online rental service for scholarly research with thousands of academic publications available at your fingertips. They present as small painless nodules in the dermis, subcutis, or mucosal surfaces.
It is also synonymously known as gingival granular cell tumor gct of the newborn and was first described by newmann in 1871, hence also called as newmanns tumor. Granular cell tumors gcts of the neurohypophysis are rare tumors seen in the sellar and suprasellar region that are poorly understood because of the confusion regarding their nomenclature and indeterminate imaging features. Oct 25, 2016 granular cell tumors gcts are soft tissue tumors that can occur anywhere in the body. More than 98% of these tumors are benign, but 1% to 2% of all cases occur as malignant tumors. Granular cell tumors gcts tend to present as solitary nodules, not uncommonly affecting the dorsum of the tongue but also involving the skin, breasts, and internal organs. Later, gca was found to be a highly aggressive neoplasm, in dramatic contrast to the benign. To the best of our knowledge, only two genuine cases of granular cell tumour have been described in the thyroid gland. Malignant granular cell tumor mgct is a rare highgrade mesenchymal tumor of schwann cell origin. The tumour cells are large in size, with small, uniform.
They are not reactive and typically need surgery for treatment. Most granular cell tumors are found in the skin or the mouth, but they are uncommon even in those places. Granular cell tumors of the breast american cancer society. Mgcts from the armed forces institute of pathology, with a mean age of 40 years. Histologic and immunohistochemical studies of granular. The granular cell tumor in the lung of the horse and the intracranial meningioma in a dog reacted to the antibody sl00 protein. Rarely, the tumor can occur as multiple nodules too. There were 11 female and eight male esophageal gct patients with a median age of 42. Most tend to develop between the third and fifth decades of life. Granular cell tumour of the thyroid gland granular cell tumour of the thyroid gland milias, s.
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