Pdf granules in granular cell lesions of the head and. Generally, 90% of patient survived if diagnosed and treated at an early stage. Spindle cell carcinoma of the parotid gland cambridge core. The majority of ovarian spindle cell lesions fall into the broad category of fibromatous neoplasms whereas others in the sex cordstromal group may also be composed of spindle cells, including thecomas, granulosa, and sertolileydig cell tumors and rarer neoplasms, such as sclerosing stromal tumor and signetring stromal tumor. The tumors generally begin in layers of connective tissue such as that under the skin, between muscles, and surrounding organs, and will generally start as a small lump with inflammation that grows.
Pdf granules in granular cell lesions of the head and neck. Spindle cell neoplasms arising in the skin comprise a heterogeneous group of tumors with divergent lineages. Jun 29, 2007 a diagnosis of primary spindle cell carcinoma of the parotid gland was made. Spindle cell carcinoma spcc is an uncommon head and neck squamous cell carcinoma scc variant consisting of spindled and or pleomorphic cells with epithelial differentiation. Jan 15, 2019 spindle cell squamous cell carcinoma of skin may appear as skin lesions, commonly on sunexposed areas, mostly the head and neck region. Immunohistochemical studies revealed keratin positivity and vimentin negativity in the squamous cell carcinoma component. Spindle cell melanoma scm is a morphologic variant of melanoma 1. In the oral cavity, the origin of the spindle cell neoplasms may be traced to epithelial. Cutaneous spindle cell neoplasms are relatively common and present surgical pathologists with diagnostic challenges.
In fact, head and neck sarcomas represent an estimated 15% of sarcomas in adults and 35% of sarcomas in children 16. Cutaneous spindle cell neoplasms are often diagnostically. Spindle cell sarcoma is a type of connective tissue cancer in which the cells are spindleshaped when examined under a microscope. Distinctive soft tissue tumors of the head and neck. A case report of spindle cell sarcomatoid carcinoma of the. The presentation and clinical history of patients presenting with head and neck lesions can be used to guide further imaging, which can provide important diagnostic and therapeutic. Spindle cell rhabdomyosarcoma of the anterior mandible.
Diagnosis of this spindle cell sarcoma is conducted by multiple tests, such as xray, mri scan, bone scan and biopsy. Clinical, pathologic, and biologic implications of these lesions when they arise in the mucosae of the upper aerodigestive tracts of the head and neck are presented. The intriguing array of spindle cell lesions occurring, especially in the head and neck region, poses a critical diagnostic challenge not only to the histopathologist but also ultimately to the clinicians for planning an appropriate treatment protocol. Jun 10, 2019 approximately 1520% of sarcomas occur within the head and neck. Some head and neck squamous cell carcinoma variants can be human papillomavirus hpvrelated and have improved outcomes. Spindle cell carcinomas are staged according to the tnm. Clinicopathological and immunohistochemical analysis of spindle.
Sarcomatoid spindle cell carcinomas of the head and neck are unusual variants of squamous carcinoma commonly reported in the larynx but also described in other mucosal sites such as gingiva, tongue, hypopharynx and nasal cavity 1 2345678910111214151617. Research article open access cytological diagnosis of. Two children are described who presented with signs and symptoms consistent with rhabdomyosarcoma, but who were found to hav e benign spindle cell lesions. Moreover, in this specific district, fs has to be differentiated from spindle. Examples include dermatofibrosarcoma protuberans, desmoplastic melanoma, malignant fibrous histiocytoma, leiomyosarcoma, malignant peripheral nerve sheath tumor, and spindle cell squamous cell carcinoma. Epidermal growth factor receptor egfr is expressed by 90 % of conventional scc, and high level expression is associated with a poorer prognosis.
Fs has also been reported in the head and neck areas and, as in other districts, it has to be differentiated form other benign, locally aggressive and malignant spindle cell tumors. For biopsies from the head and neck assessment is further complicated. Sarcomatoid spindle cell carcinoma of the head and neck. The term spindle cell lesion, or tumor, is a purely descriptive one and if applied without further qualification is meaningless as a guide to therapy and. Many patients evaluated for head and neck fnas have had prior surgery or chemoradiation treatment a variety of benign and malignant mesenchymal neoplasms involve the soft tissues of the head and neck thus a common diagnostic dilemma occurs.
The study is based on 140 cases of spindlecell tumor of the gastrointestinal tract seen at the massachusetts general hospital. Of the total of 171 cases of mucosal spindle cell tumors of the head and neck, cases of sarcomatoid carcinoma were diagnosed and selected as per the diagnostic algorithm fig. Since there is a lower incidence of this neoplasm, histopathological diagnosis can be challenging, especially in the oral region 8. In the most recent world health organization who system for classification of soft tissue tumors, which was published in 2002, soft tissue. Spindle cell carcinoma spcc is a rare and unusual biphasic. Spindle cell hemangioma sch is a unique benign vascular lesion. We provide an overview of the lesions with a spindle cell morphology that may be encountered in this region and detail a histological. These neoplasms account for less than 1% of neoplasms occurring in the oral cavity 7. A diagnosis of primary spindle cell carcinoma of the parotid gland was made. It is most frequently encountered in the larynx, and also occurs in the nasal cavity, hypopharynx, oral cavities, esophagus, trachea, skin and breast. May 28, 2008 spindle cell lesions of the head and neck are quite diverse with great clinical and biological heterogeneity. Nov 30, 2017 spindle cell hemangioma sch is a unique benign vascular lesion. Spindle cell hemangioendothelioma mdedge dermatology.
One challenging feature of head and neck pathology is that a dizzying array of spindle cell lesions occur here ranging all the way from reactive. Synchronous glottic granular cell tumor and subglottic. Spindle cell carcinoma of the oral cavity and oropharynx. Examples include dermatofibrosarcoma protuberans, desmoplastic melanoma, malignant fibrous histiocytoma, leiomyosarcoma, malignant peripheral nerve sheath tumor, and spindle cell squamous cell. Immunohistochemistry along with routine histopathology is essential in establishing the diagnosis of spindle cell. Spindle cell neoplasms are defined as neoplasms that consist of spindleshaped cells in the histopathology. Other soft tissue lesions that show a notable tendency to occur in the head and neck region, albeit many of them rare, include spindle cellpleomorphic lipoma, a relatively common tumor most often. For biopsies from the head and neck assessment is further complicated by the range of tissues present at this site, and the limited amount of material which may be available for study. Spindle cell sarcoma symptoms, prognosis, survival rate. Other soft tissue lesions that show a notable tendency to occur in the head and neck region, albeit many of them rare, include spindle cell pleomorphic lipoma, a relatively common tumor most often. This is one of the few series devoted to head and neck ks showing high prevalence of hivpositivity, but also unusual presentations in hivnegative patients with primary origin in the skin of the ear and the auditory canal. Request pdf spindle cell lesions of the head and neck. Spindle cell carcinoma of the head and neck makes up 3% of all squamous carcinomas in the region and may occur at any site but is most common in the larynx. To our knowledge, this is the first report of synchronous granular cell tumor and subglottic spindle cell carcinoma.
The important diagnostic issues include 1 distinguishing spindle cell squamous cell carcinoma and malignant melanoma from true sarcoma, 2 discriminating between benign spindle cell lesions and malignancies, and 3 classifying histologic types and subtypes. Aug 22, 2010 distribution of spindle cell lesions in head and neck, 20042010 n 171 clinical information in terms of sociodemographic profile and site of lesion was available in all the 103 patients which were taken from the hospital records. Spindle cell hemangioma in the mucosa of the upper lip. Spindle cell lesions of the head and neck diagnostic histopathology. Stage ii sarcoma has 81% of survival while stage iii has 56% survival rate. Many patients evaluated for head and neck fnas have had prior surgery or chemoradiation treatment a variety of benign and malignant mesenchymal neoplasms involve the soft tissues of the head and neck thus a common diagnostic dilemma occurs with respect to spindle cell lesions. A majority of these lesions are cutaneous and congenital. Spindle cell carcinomas of the head and neck rarely harbor. Unlike the development of squamous cell carcinoma, the development of sarcomas is unrelated to smoking and alcohol use. Pigmented lesions of the head and neck t here is a wide spectrum of pigmented lesions which present an oral mucosa or skin in the head and neck area. In the oral cavity some of the pigmented lesions are physiologic, but most are considered pathologic. Spindle cell lesions of the head and neck are quite diverse with great clinical and biological heterogeneity. Spindle cell lesions in the head and neck region have higher site predilection to skin, scalp soft tissue, orbit, neck and the upper aerodigestive tract 8.
Epidermal growth factor receptor expression in spindle. It usually presents as a polypoid mass and often at an early stage due to local obstruc tive symptoms. Spindle cell lipoma scl is an uncommon benign lipomatous tumor, most commonly occurring in the posterior neck and shoulder. The spindle cell neoplasms of the oral cavity ncbi. Spindle cell squamous carcinoma sarcomatoid carcinoma. It is now believed that spindle cell he is most likely a benign reactive process that develops because of aberrations in local blood flow,36 which perkins and weiss3 have restudied and recommended that the entity be renamed spindle cell he for solitary lesions and spindle cell hemangiomatosis for multifocal lesions.
The rate of survival is extremely low if the sarcoma has metastasized to other parts of the body such as in the case of stage iv spindle cell sarcoma. The study is based on 140 cases of spindle cell tumor of the gastrointestinal tract seen at the massachusetts general hospital. Spindle cell lesions can occur in head and neck skin, in the soft tissues of the scalp, orbit, and neck, and along the upper aerodigestive tract uadt mucosa. Some are malignant while many others are benign or simply reactive in nature. For the 19 sarcomatoid carcinomas spindle cell carcinomas, all four markers stained the spindle cell component of some of the tumors see table 2 and figures 1 and 2. Spindle cell squamous cell carcinoma of skin may appear as skin lesions, commonly on sunexposed areas, mostly the head and neck region. Spindle cell lipomas scls are benign lipomatous neoplasms that classically arise in the posterior neck, upper back, and shoulders of older male patients. Granular cell tumors gcts are unusual benign tumors that can appear anywhere in the body but tend to occur in the subcutaneous tissues of the head and neck, where the tongue followed by the larynx are the most common sites. Although it usually arises within subcutaneous tissues, it may also occur rarely in the.
Spindle cell carcinoma spcc is an uncommon head and neck squamous cell carcinoma scc variant consisting of spindled andor pleomorphic cells with epithelial differentiation. The purpose of this study was to investigate the ct and mr imaging features of scl in the head and neck. In spindle cell sarcoma, cells present in the connective tissue beneath the skin, adjacent areas of the organs, and the muscles begin to create tiny bumps leading to progression of tumor. We present a case of sch in the upper lip of a 41yearold woman. Immunohistochemistry along with routine histopathology is essential in establishing the diag.
Spindle cell carcinoma spcc or sarcomatoid carcinoma is a highly malignant variant of squamous cell carcinoma which comprises 2% to 3% of all laryngeal cancers. Spindle cell lipomas arising at atypical locations. Spindle cell lesions of head and neck include a diverse group of clinically and biologically heterogeneous lesions. Spindle cell carcinoma of head and neck, a subtype of squamous cell carcinoma is a unique and rare neoplasm. Spindle cell carcinoma spcc, also known as sarcomatoid carcinoma or pseudosarcoma, of head and neck is a rare neoplasm. For biopsies from the head and neck assessment is further complicated by the range of tissues present at this site, and. Cutaneous spindle cell neoplasms patternbased diagnostic approach joon hyuk choi, md, phd. Sarcomatoid spindle cell carcinomas of the head and neck are unusual variants of squamous carcinoma commonly reported in the larynx but also described in other mucosal sites such as gingiva, tongue, hypopharynx and nasal cavity 117.
Immunohistochemistry ihc was performed on the representative paraffin blocks in 82 of the 103 cases. It is considered to be a biphasic tumor that is composed of a squamous cell carcinoma in situ or invasive and spindle cell carcinoma with sarcomatous appearance. It occurs in all age groups but more often in young adults. A case report of spindle cell sarcomatoid carcinoma of. These tumors have now been proved to be monoclonal, evolving from conventional squamous carcinoma with. Ks should be included in the differential diagnosis of difficulttoclassify spindle cell lesions at this uncommon location. Ct n 5 and mr n 3 images of seven patients five men and two women. We provide an overview of the lesions with a spindle cell morphology that may be encountered in this region and detail a. We sought to characterize the occurrence of this entity at nonclassic sites.
Most spindle cell lipomas arising at atypical sites showed immunoreactivity for cd34 a, lack of desmin staining b, and loss of rb nuclear expression in tumor cells endothelial cells show normal intact staining for rb c. These masses typically arise in males aged 4070 years and originate most frequently in the subcutaneous tissue of the upper back, posterior neck, and shoulders. Tumors of each histologic type may be further subclassified on the basis of their biologic behavior as benign, intermediate ie, having malignant potential, or malignant. The fibrous capsule of the lesions will show positive staining for epithelial membrane. An associated inflammatory component may be present, and this can become more prominent as lesions mature. Spindle cell carcinoma spcc, also known as sarcomatoid carcinoma, is a rare malignancy of the head and neck regions. All cases of scl arising at atypical sites were retrieved from our archives. It has a more aggressive behavior as compared to classical squomous cell carcinoma warranting surgical interventions with wider surgical margins. Imaging plays an important role in the noninvasive diagnosis and characterization of nonepithelial soft tissue tumors of the head and neck, providing clues about tumor grade. Cutaneous spindle cell carcinoma describes a heterogenous group of tumors that share an atypical spindle cell morphology and infiltrative growth pattern. Spcc is known as a high malignant variant of squamous cell carcinoma, which is composed of conventional squamous cell carcinoma component, either insitu andor invasive and malignant spindle component with sarcomatous. Scm is a diagnostic challenge for pathologists, because there are many lesions which can be spindle cell rich and scm lack of classic conventional melanoma characteristic 2. For spindle cell carcinomas with poorly differentiated epithelial tumor components lewis et al. Spindle cell lesions in the head and neck region have higher site predilection to.
A diagnostically challenging spindle cell carcinoma of the. Fibrosarcomas represent the other end of the spindle cell lesion spectrum in that they are soft tissue malignancies of fibroblastic origin. A number of entities arising in the head and neck region can fall into this category, but the most common are. The mass was elastic firm, mobile, bluish in color, and well demarcated in. The mass developed 5 years earlier and enlarged after repeated ethanol injections. Clinical, pathologic, and biologic implications of these lesions when they arise in the mucosae of the upper aerodigestive tracts of. For biopsies from the head and neck assessment is further complicated by the range of tissues present at this site, and the limited amount of.
Distinctive soft tissue tumors of the head and neck modern. Spindle cell lipomas arising at atypical locations american. The presentation and clinical history of patients presenting with head and neck lesions can be used to guide further imaging, which can provide important diagnostic and therapeutic considerations. It usually presents as a polypoid mass and often at an early stage due to local obstructive symptoms. The material was culled from 12,000 autopsy protocols covering the years since 1900 and from 5,3 records of gastrointestinal tumors histopathologically proved at operation since january 1931.
Epidermal growth factor receptor expression in spindle cell. The lesions may ulcerate and cause scarring of skin the treatment of choice is a surgical excision with clear margins followed by radiation therapy or chemotherapy, as decided by the healthcare provider. The term spindle cell lesion, or tumor, is a purely descriptive one and if applied without further qualification is meaningless as a guide to therapy and prognosis. In a previous series of laryngeal malignancy, about 1%. Pigmented lesions of the head and neck pdf free pdf epub. Granular cell lesions of the oral mucosa, jaws, and salivary glands constitute a heterogeneous group of lesions which may be either odontogenic, salivary gland, or metastatic in origin.
The head and neck is an embryologically and anatomically complex area, with a number of important structures and organs lying in close proximity to one another. The mass was elastic firm, mobile, bluish in color, and well. Solitary painless cutaneoussubcutaneousmucosal nodule, head and neck or lower extremity of adults well circumscribed but unencapsulated multinodular, biphasic tumors primitive cellular proliferation of small short spindle cells with thin walled branching blood vessels and whorled nodulesfascicles of plump spindle cells. The diagnosis of lesions with spindle cell morphology is often difficult. Spindlecell tumors of the gastrointestinal tract radiology.