Spindle Cell Squamous Carcinoma Research Articles

Leiomyosarcoma of the esophagus, arising from lamina muscularis mucosae, in a combination with microinvasive squamous cell carcinoma-A case report.

Leiomyosarcoma of the esophagus is a very rare disease, accounting for less than 0.5% of malignant esophageal tumors. Esophageal leiomyosarcoma combined with squamous cell carcinoma is even rarer than solitary leiomyosarcoma. To our knowledge, there are less than ten cases of simultaneously diagnosed leiomyosarcoma and squamous cell carcinoma of the esophagus. A 66-year-old male was admitted to our hospital suffering from epigastric pain, asthenia, weight loss, and difficulties when feeding with solid food which had been present for 2 weeks. A computed tomography scan showed a large tumor tissue mass in the mid-to-distal part of the esophagus. The patient underwent robot-assisted surgery-an esophagectomy with esophagogastrostomy. Histologically, the tumor consisted of highly pleomorphic spindle cells with multiple atypical mitosis and necrotic areas. An immunohistochemical examination was performed to distinguish leiomyosarcoma from spindle cell squamous carcinoma or malignant GIST. Tumor cells stained diffusely positive for smooth muscle actin, but negative for p63, CD117, and CKAE1/AE3. Tumor invasion involved mucosa and submucosa, without tunica muscularis propria. Microinvasive well-differentiated squamous cell carcinoma was also noted in the mucosa at the borders between the tumor and the healthy part of the esophagus. The aim of the manuscript is to present an extremely rare case of combined polypoid leiomyosarcoma and microinvasive squamous cell carcinoma of the esophagus, cured by robot-assisted surgical intervention and to emphasize that such cases should be examined carefully, including additional diagnostic tests such as Immunohistochemistry (IHC) in order to define the correct diagnosis.

Granulation tissue-like spindle cell (sarcomatoid) carcinoma of the head and neck: a deceptively bland-looking underdiagnosed malignancy

The diagnosis of head and neck spindle cell squamous carcinoma (SC-SCC) is often challenging. Lesions with a prominent inflammatory infiltrate and reactive vessels may have a granulation tissue-like appearance, therefore being difficult to distinguish from reactive lesions, like contact ulcers, post-intubation granulomas, inflammatory pseudotumors, or benign vascular lesions. In this study, we analyzed the clinicopathological features of a series of 17 head and neck SC-SCC with granulation tissue-like appearance. All patients, but two, were males, ranging in age between 57 and 80 years. The larynx was the most frequently affected site (n = 12), followed by the tongue (n = 4). One tumor was hypopharyngeal. Most consult cases were submitted with benign suggestion or because of unexpected recurrences of granulation tissue polyps. Histologically, all lesions consisted of an ulcerated polypoid proliferation of moderately to markedly atypical spindle cells, with a minor component of conventional invasive or in situ squamous carcinoma. At least one cytokeratin cocktail was positive in 13 cases. The staining was limited to a few neoplastic cells in most cases. Positivity for p63, p40, and cytokeratins 5/6 was detected only in the conventional squamous cell carcinoma component, when present. ALK1 was negative in all cases. Sixteen cases were tested for p53 and all showed aberrant expression (12 diffusely positive and 4 of null-phenotype). The diagnosis of granulation tissue-like SC-SCC is challenging due to the close clinical and histological overlap with several benign conditions. Since the expression of epithelial markers is limited, the use of an immunohistochemical panel including p53 is recommended.

High-grade Spindle Cell Squamous Carcinoma of the Supraglottic Larynx Treated with Definitive Curative Radiation Therapy- A Rare Case Report

Spindle cell carcinoma or sarcomatoid carcinoma is a highly malignant form of squamous cell carcinoma with a low incidence of 2-3% in the larynx. Considering the poor prognosis, early diagnosis and treatment are imperative. This case report describes a high-grade spindle cell carcinoma of the supraglottic larynx treated with definitive radiation therapy.

SATB2 Immunopositivity in Spindle Cell (Sarcomatoid) Squamous Cell Carcinoma: A Potential Pitfall in Diagnosis.

Spindle cell squamous cell carcinomas (SpSCC) are aggressive neoplasms constituting 1% of oral cavity tumors. A proportion of SpSCC do not stain with epithelial markers, and frequently express mesenchymal markers, viz. Vimentin, smooth muscle actin, muscle specific actin, S100 and desmin, confounding the diagnosis. Immunoexpression of SATB2, a transcription factor indicating osteoblastic lineage, has not been evaluated in SpSCC previously. We therefore performed SATB2 immunohistochemistry in 15 cases of SpSCCs and scored them with respect to intensity and percentage of tumor cells stained. SATB2 immunopositivity was identified in 9/15 (60%) SpSCCs, with varying intensity and distribution. Eight cases (53.3%) showed nonfocal staining of moderate to strong intensity, and 1 case (6.7%) showed focal weak staining. Of these, 3 cases (3/9; 33.33%) did not stain with epithelial/squamous markers. Thus, a subset of SpSCC demonstrate SATB2 immunopositivity. In oral tumors with bone involvement, SATB2 positivity may lead away from the diagnosis of SpSCC. Knowledge of this aberrant immunostaining is, therefore, extremely relevant to guard against misdiagnosis as osteosarcoma, particularly on biopsies which lack adjacent dysplastic epithelium, in cases which are monophasic spindle cell, and in those that do not show immunopositivity for epithelial/ squamous markers. Our results emphasize that an appropriate panel and not a single immunomarker is required to distinguish SpSCC from mesenchymal tumors including osteosarcoma.

Second Primary Head and Neck Malignancies in Patients With Prior Human Papilloma Virus (HPV) Associated Oropharyngeal Squamous Cell Carcinoma (OPSCC) Treated With Radiation

The outcomes of second primary head and neck malignancies in survivors of HPV associated OPSCC treated with radiation have not been well documented. The purpose of this study was to review the rate, management, and outcomes of non-metastatic second head and neck malignancies in patients with a history of HPV positive OPSCC treated with definitive or postoperative radiation (RT).This is a single institution IRB approved retrospective study of patients with HPV positive OPSCC treated from 2001-2017 with definitive radiation and at least 3 years of follow up. Diagnoses were considered to be a second head and neck malignancy if they were distinct from index OPSCC with the exclusion of a primary or regional recurrence, primary site > 2 cm from the index OPSCC, and occurred at least 2 years from prior RT. Patients with second malignancies involving the thyroid were excluded. Actuarial analysis was used to calculate overall survival and disease control rates.There were a total of 418 patients who met inclusion criteria with a median follow up of 88.5 months (50.9-150.9 months). A total of 11 patients (2.6%) developed a second primary malignancy in the head and neck (2 larynx, 1 nasal cavity, 6 oral cavity and 2 oropharynx) and all were treated with definitive surgery. Median time between first and second cancers was 73 months (25-123 months). Median follow up for these patients was 25.4 months (7.7-69 months) and the median age at second malignancy was 65 years (range 54-73 years). 64% of patients were former smokers (quit > 3 months) and 36% were never smokers. Pathological tumor stage of the second cancer was T1 in 36%, T2 in 27%, T4a in 36%. The histology was squamous cell carcinoma in 9 patients, spindle cell squamous carcinoma in 1 patient, and adenosquamous carcinoma 1 patient. HPV status was negative in 45.5%, positive in 18.2% and unknown in 36.4%. Post-operative re-irradiation was given in 55%, of which 50% received concurrent chemotherapy. Locoregional control rates at 12 and 24 months were 100% and 62.5% (95% CI: 28.9-96.1%), respectively. Of the 2 patients with local failure, which occurred at 18 and 33 months after diagnosis, salvage therapy with surgery was successful. Regional failure was diagnosed in 2 patients at 13 and 16 months after diagnosis, and both of these patients ultimately died as a result of regional failure. There were no distant failures. One additional patient had died at last follow up which was unrelated to cancer. Estimated 2-year overall survival from the second head and neck malignancy was 78.9% (95% CI: 53.0-100%).The risk of a second head and neck malignancy in survivors of HPV associated OPSCC is low, but as the incidence of HPV related OPSCC remains elevated and the likelihood of cure is excellent, secondary malignancy is an important long-term risk. Despite prior RT, consideration of post-operative re-irradiation is important as regional failure may carry a poor prognosis.

Anaplastic spindle cell squamous carcinoma arising from classical papillary thyroid carcinoma with foci of columnar cell component: A case report

We report a case of the anaplastic spindle cell squamous carcinoma(SC) arising from classical papillary thyroid carcinoma(PTC) with foci of columnar cell component in a 69-year-old Chinese woman. The tumor of the right lobe was composed of spindle cell SC and PTC. Histologically,the spindle cell SC were arranged into bundles with marked cellular atypia, nuclear pleomorphism and more mitotic figures. The tumor of the left lobe was a PTC without any SC elements,which was composed of two components, namely classical and columnar cell variants PTC. The columnar cell elements occupied more than 20 % of PTC, which showed striking stratification of nuclei with no characteristic nuclei for the classical type of PTC. The patient has not received any other treatment after radical surgery. She had no tumor recurrence and other complications 13 months after operation. Only classical PTC but not columnar cell PTC and SC had been identified in 7 out of 50 excised cervical lymph nodes. In order to clarify the origin of PTC and spindle cell SC, we performed the BRAFV600E(c.1799 T > A) mutational analysis. The results of molecular analysis showed that BRAFV600E(c.1799 T > A) mutation existed in both components, which further confirmed that spindle cell SC was transformed from PTC.

S2129 Who’s the Father?: The Duality of Esophageal Spindle-Cell Squamous Carcinoma

Introduction: Spindle-cell squamous carcinoma (SpCC) is an extremely rare variant of SCC previously described in literature with a series of confounding names as a result of its intriguing yet confusing origin. Furthermore, the rarity of SpCC along its dual cellular component has led to diagnostic challenges of this highly aggressive entity. Herein a curious case of an African American male with atypical chest pain diagnosed with spindle-cell squamous carcinoma of the esophagus. Case Description/Methods: A 53-year-old male with history of tobacco and alcohol abuse presented to ER with atypical chest pain and questioning revealed epigastric pain, worsening heartburn, weight lost, and difficulty swallowing since 3 months ago. EKG without evidence of acute ischemia. Physical exam and labs unremarkable. CT chest with evidence of mid-to-distal esophageal distention with associated distal nodular lesion of 1.5 cm in size. EGD revealed distal esophageal bulky polypoid lesion. Biopsy suggested spindle cell malignancy, yet inconclusive due to scant amount of tissue. Second EGD with evidence of friable lesion obstructing 75% of esophageal lumen. Biopsy revealed expression of P40, P63, Ck5/6 and morphology suggestive of SpCC. Patient referred to Oncology service and chemotherapy-radiotherapy started with remission of disease. A year later patient presented with severe dysphagia. Initial EGD negative for strictures, tumor, or fistula. Bronchoscopy negative for fistulization. Due to worsening dysphagia, patient re-admitted and CT chest concerning for fistulous tract. New EGD demonstrated tracheoesophageal fistula at 31 cm and AGILE stent placed with improvement of dysphagia. Discussion: SpCC is a rare subtype of SCC but with similar risk factors such as tobacco use and alcohol consumption. The spindle cell component simulating a sarcoma and a non-specific clinical presentation precludes a correct diagnosis. Our patient initially presented with atypical chest pain, and review of systems revealed symptoms of progressive dysphagia and weight loss which guided the diagnosis. A second biopsy performed, and several immunohistochemistry tests confirmed the presence of both a carcinomatous and sarcomatous component. As a result, our case is an example of one of the rarest malignancies affecting the esophagus and its complications. It also highlights the importance of a thorough history taking, adequate tissue sampling, use of staining techniques to reach a correct diagnosis, as well as incorporation of innovative medical devices.Figure 1.: Spindle-cell squamous carcinoma lesion with tracheoesophageal fistula complication and subsequent Agile Stent placement.

Maxillary Sarcomatoid Carcinoma with Rhabdo-Myosarcomatous Dedifferentiation: a Clinic-Pathological Emphasis.

Spindle cell carcinoma or sarcomatoid carcinoma (SC) has been recently defined as a monoclonal dedifferentiated form of conventional squamous cell carcinoma (SCC) and termed as spindle cell (sarcomatoid) squamous carcinoma as per the current World Health Organization (WHO) classification. Although the frequency of SC of the breast, larynx, esophagus, and lungs has been reported, the frequency of oral SC with rhabdo-myosarcomatous dedifferentiation of the maxilla is unknown yet because of the less number of reported cases. Microscopic features of spindle cell carcinoma resemble many benign and malignant oral lesions. Therefore, a careful depiction with the help of routine hematoxylin and eosin along with immunohistochemistry using appropriate markers is necessary to diagnose spindle cell carcinoma. SC is itself a rare entity accounting for <1% of SCC; however, rhabdo-myosarcomatous dedifferentiation is exceptional; therefore, our aim is to highlight this extremely rare entity. This article focuses on SC with rhabdo-myosarcomatous dedifferentiation; other than rhabdomyosarcoma, with an emphasis on strategies to resolve the diagnostic dilemmas these tumors may present.

The Most Common Mistake in Laryngeal Pathology and How to Avoid it

Upper aerodigestive tract (UADT) spindle cell squamous carcinoma (SCSC), also known as sarcomatoid carcinoma, is a high-grade subtype of conventional squamous cell carcinoma (SCC) that is histologically characterized by a combination of differentiated SCC in the form of intraepithelial dysplasia and/or invasive differentiated SCC, and the presence of an invasive (submucosal) undifferentiated malignant spindle-shaped and pleomorphic (epithelioid) cell component. Typically, SCSC presents as a superficial polypoid mass not infrequently with surface ulceration precluding identification of an intraepithelial dysplasia. Further, in many cases an invasive differentiated SCC is not identified. Adding to the complexity in such cases, is that immunohistochemical staining in a significant minority of cases is negative for epithelial-related markers but often the cells express mesenchymal-related markers. In such cases, differentiating SCSC from a reactive (benign) spindle cell proliferation or a mucosal-based sarcoma can be problematic, with treatment implications. Herein, we detail the clinical and pathologic features of laryngeal SCSC and discuss the rationale for diagnosing a carcinoma and avoiding a diagnosis of sarcoma. In our experience, such cases represent one of the more common mistakes made in laryngeal pathology. Yet, virtually all such lesions are SCSCs. The treatment and prognosis relies on the accuracy of this distinction.

SUN-487 A Case of Type 2 Anaplastic Spindle Cell Squamous Cancer

Background: Papillary thyroid carcinoma (PTC) is generally considered a relatively slow-growing, indolent cancer; however, an important subset of these cancers known as the tall cell variant (TCV), portend a more aggressive prognosis. TCV PTC is characterized by cells, which are at least twice as tall as they are wide. Patients diagnosed with TCV PTC fare worse than those with the classic variant. A less common thyroid cancer, spindle cell squamous cell carcinoma (SCSC), may arise as a primary tumor, but rarely also alongside or from a previous TCV PTC (1). It may also occur as a component of an anaplastic thyroid cancer, the most aggressive and rarest thyroid malignancy. There are three main types of anaplastic SCSC arising from TCV PTC based on histological characteristics: Type 1 is defined by the presence of both TCV and SCSC within the initial resection; type 2 refers to when the SCSC arises as a recurrence or metastasis in patients with a known history of TCV PTC; type 3 occurs when SCSC presents as a primary laryngeal SCC in patients with or without a known history of TCV. We report a case of type 2 anaplastic SCSC. Clinical Case: A 76 yr old man with hx of invasive TCV PTC (diagnosed 2011) s/p total thyroidectomy with b/l neck dissection and laryngectomy, as well as 200 mci of I-131 (in 2012), returns to clinic in 2019, with worsening unexplained weight loss (> 20 lbs). He had been lost to follow-up since 2015. Now, chest CT showed a new, necrotic, left lower lobe mass obstructing the bronchus, as well as bony erosion of the right clavicle and manubrium. Bronchoscopy of the mass was performed, and pathology was consistent with metastatic anaplastic thyroid cancer with squamous nests. Immunohistochemistry was positive for PAX-8, p40, p63 and negative for TTF-1, Napsin A, TG and PDL-1. The endobronchial tumor tissue was compared to the prior resection specimen from 2011, showing morphologic similarity to the squamoid nests. In retrospect, these nests likely represented small foci of anaplastic thyroid cancer arising in association with TCV PTC. The positive staining for PAX-8 and lack of expression for TTF-1 and Napsin A supports this interpretation and is evidence against a primary pulmonary squamous cell carcinoma. Next Gen Sequencing was positive for BRAF V600E mutation, which allowed us to offer BRAF inhibitor and MEK inhibitor therapy. Unfortunately, due to patient’s non-adherence to therapy and follow-up clinic visits, we are not able to assess his response. Conclusion: This is a case of the rarely described type 2 anaplastic SCSC and is an example of the very poor prognosis associated with TCV PTC. It is a reminder that this variant of PTC should be treated more aggressively from the time it is first identified on biopsy. Reference: (1) Gopal PP et al. The variable presentations of anaplastic spindle cell squamous carcinoma associated with tall cell variant of papillary thyroid carcinoma. Thyroid. 2011;21(5):493–499.

A rare case of bipartite combined tumour of the oesophagus

BackgroundBipartite combined oesophageal tumours are an exceedingly rare entity and much less is known about the natural history of these tumours following curative surgery. The authors present a case of a bipartite combined oesophageal tumour comprising of sarcomatoid carcinoma and small cell carcinoma with early postoperative recurrence.Case presentationA 63-year-old Chinese male with a smoking history presents with hemoptysis on a background of dysphagia and odynophagia for 1 month. An endoscopic evaluation found an exophytic oesophageal tumour with contact bleeding for which biopsy of this lesion returned as a malignant high-grade tumour where immunohistochemistry staining was unable to establish the lineage of the tumour. Differential diagnoses include sarcomatoid carcinoma and malignant undifferentiated sarcoma. With the provisional diagnosis of a high-grade oesopheageal sarcoma, the patient underwent minimally invasive McKeown’s oesophagectomy. Final histological assessment was pT1bN0 with two histological types of malignancy within a single tumour—70% poorly differentiated spindle cell squamous carcinoma and small cell carcinoma. He was planned for adjuvant chemotherapy in view of the small cell carcinoma component after the resolution of the postoperative infective collections. A computed tomographic scan performed 4 months postoperatively demonstrated metastasis to the lung, pleura, thoracic nodes and liver. Biopsy of the largest lung nodule confirmed small cell neuroendocrine carcinoma with features similar to the small cell carcinoma component in the prior oesophagectomy specimen. He was thereafter initiated on palliative chemotherapy aimed at three weekly carboplatin and etoposide aimed at a total of 4 cycles with peglasta support. Etoposide was stopped during the first cycle due to asymptomatic bradycardia. The regime was then converted to carboplatin with irinotecan for 5 cycles. Repeat computed tomographic scan performed 3 weeks after the completion of chemotherapy showed a complete response of lung and liver metastasis and no evidence of local recurrence or distant metastasis.ConclusionThe management of bipartite combined oesophageal tumours should be guided by its more aggressive component. Bipartite combined oesophageal tumours with a small cell carcinoma component are believed to demonstrate aggressive tumour biology likened to that of primary oesophageal small cell carcinoma. Preoperative confirmation of a combined tumour may be challenging, and biopsy results may only yield one of the two components. The more aggressive component is usually a small cell carcinoma, for which the mainstay of therapy is platinum-based chemotherapy rather than surgery.

Spindle Cell Squamous Carcinoma of the Esophagus: A Rare Malignancy With Biphasic Morphology

Introduction Spindle-cell squamous carcinoma (SpCC) of the esophagus is rare and accounts for 0.1%-1.5% of all esophageal tumors. Here we report a rare case of SpCC in a patient with human immunodeficiency virus (HIV). Case Description A 59-year-old male with a past medical history of HIV (undetectable viral load, CD4+ 491) presented with a 30 pound unintentional weight loss due to odynophagia over the last 2-3 months. He has a history of heavy alcohol use and smoking. He underwent an esophagogastroduodenoscopy which showed a large ulcerated obstructing mass spanning the middle and distal esophagus. Biopsies were consistent with granulation and fibrinous exudate with a rare group of atypical squamous epithelium. Cytomegalovirus and herpes simplex virus stains were negative. Due to the non-diagnostic nature of the tissue, an EUS with a core biopsy was performed and subsequent tissue was still non-diagnostic. Due to continued symptoms and rapid growth over the next few months, he underwent an esophagogastrectomy despite no definitive diagnosis. The pathologic specimen post-operation was consistent with a biphasic squamous cell carcinoma with spindle cell features. Immunohisto-chemical staining showed squamous cells to be positive for CK5/6, EMA, p63, MCK, p40, and pankeratin consistent with squamous cell carcinoma. The spindle cells were positive for vimentin. Final diagnosis was SpCC. He has since been started on adjuvant chemotherapy with FOLFOX. Discussion SpCC is a rare biphasic tumor characterized by a large pedunculated bulky mass in the middle third of the esophagus composed of malignant spindle cells and carcinomatous components. Obstructive symptoms are often a presenting sign which allows for an earlier diagnosis and better prognosis compared to squamous cell carcinoma. The spindle cells originate from the stroma while squamous cells are on the surface which may be composed of carcinoma or dysplasia. Immunohistochemical analysis is necessary for the diagnosis of both carcinomatous and sarcomatous features. Biopsy samples are often non-diagnostic because of insufficient size, depth, and they often miss the cellular components needed for diagnosis. Given the rarity of the diagnosis, there is no consensus on treatment modalities with radiation and chemotherapy. If symptomatic, surgery is often first line and is often curative if diagnosed early.1707_A Figure 1. SpCC on retroflexion with tumor extending into the cardia1707_B Figure 2. SpCC in mid esophagus

LMP1-Positive Composite Tumour of Larynx: A Diagnostic Quandary

Malignant spindle cell tumours of the larynx are rare, of which the most common are Spindle Cell Squamous Carcinomas (SCSCs). Sarcomas are extremely infrequent in the larynx. Composite tumours, further, are quite unheard of, except for rare case reports. This case report describes a spindle cell malignancy of the larynx in an elderly male, which posed a diagnostic difficulty due to its unique morphological and immunohistochemical features. The tumour was superficial, polypoidal with the proliferation of malignant spindle cells and abundant mitoses under an ulcerated epithelium. Occasional squamous cell rests with keratin pearls were present. Spindle cells were strongly immunoreactive for vimentin, smooth muscle actin and squamoid cells were p63 positive. Desmin was negative. The dilemma in diagnosis was of a SCSC with smooth muscle differentiation against a composite tumour. The tumour also turned out to be positive for Epstein Barr Virus Latent Membrane Protein-1 (EBV-LMP1), establishing a noteworthy association.

術前化学療法により扁平上皮癌から形質転換が疑われた舌紡錘細胞癌の1例

There have been few reports on cases of spindle cell squamous cell carcinoma of the oral cavity. In this paper, we report a case of spindle cell squamous carcinoma arising in the tongue. A 76-year-old male with tongue squamous cell carcinoma was referred to our hospital. After two courses of neoadjuvant chemotherapy, subtotal resection of the tongue, unilateral radical neck dissection, and reconstruction with free transverse rectus abdominis myocutaneous flap were performed. Pathological diagnosis of the surgical specimen was spindle cell squamous carcinoma （pT3N0M0, Stage Ⅲ）. Recurrence of primary tumor was detected about four months after the operation and it showed extremely rapid growth. Palliative radiotherapy （two courses with 24Gy/8 fractions and 20Gy/5fractions） effectively controlled the patient’s quality of life. It is thought that although spindle cell squamous carcinoma clinically shows a response to radiotherapy or chemotherapy, some tumor cells （low sensitivity cancer stem cells） may resist and survive. So, radical resection with wider surgical free margin may be essential.

Spindle cell carcinoma of the mandible: a case report.

Spindle cell carcinoma is a rare highly malignant squamous cell carcinoma. Here, we describe a case of a 74-year-old Chinese female who presented with a 2-week history of pain and swelling in the left retromolar region. Surgical resection and titanium plate prosthesis were performed and histological analysis revealed spindle squamous cell carcinoma.

Spindle Cell Squamous Carcinoma During BRAF Inhibitor Therapy for Advanced Melanoma

Spindle Cell Squamous Carcinoma During BRAF Inhibitor Therapy for Advanced Melanoma

Myogenin expression in vulvovaginal spindle cell lesions: analysis of a series of cases with an emphasis on diagnostic pitfalls

Myogenin (myf4) is a nuclear transcription factor that is considered to be a sensitive and highly specific marker for skeletal muscle differentiation. Following the identification of focal strong nuclear staining with myogenin in two fibroepithelial polyps of the lower female genital tract (the index cases), we stained a series of vulvovaginal spindle cell lesions with this marker in order to investigate how widespread myogenin staining is in these lesions. Fibroepithelial polyps (n = 13), other vulvovaginal mesenchymal lesions (n = 21) and vulval or vaginal spindle cell squamous carcinomas (n = 4) were stained for myogenin. Apart from the index cases, all of the other cases were negative, except for one vaginal spindle cell squamous carcinoma, which showed focal weak nuclear immunoreactivity. Ten of 12 embryonal rhabdomyosarcomas of the lower female genital tract were myogenin-positive, as was a single vaginal rhabdomyoma. Our study illustrates that focal myogenin immunoreactivity occurs uncommonly in fibroepithelial polyps of the lower female genital tract. This may result in diagnostic confusion and misdiagnosis as a skeletal muscle neoplasm, especially the sarcoma botryoides variant of embryonal rhabdomyosarcoma.

Superficial paramucosal clear cell sarcoma of the soft parts resembling melanoma in a 13‐year‐old boy

Clear cell sarcoma (CCS) of tendons and aponeuroses, also known as melanoma of soft parts, represents an aggressive rare malignancy that is characterized by a nested or fascicular pattern of spindled cells and a pathognomonic reciprocal translocation, t(12;22)(q13;q12), that results in the fusion of EWSR1 and ATF1 genes. Numerous recent studies have recognized the importance of a cutaneous CCS variant that can mimic a broad spectrum of entities, including spindle cell melanoma, spindle cell squamous carcinoma, cutaneous leiomyosarcoma and atypical fibroxanthoma. We report a case of a 13-year-old boy with cutaneous CCS who presented with a few months history of an asymptomatic papule on the lower lip that was suggestive of a mucocele. Biopsy of the lesion showed a wedge shaped neoplasm arranged in nests and fascicles of epithelioid- to oval-shaped cells with pale cytoplasm, open chromatin and prominent nucleolus. The superficial component was closely opposed to the basal epithelium resembling the junctional nests of a melanocytic neoplasm. The process extended into and involved the striated muscle of the lip. The cells expressed S-100, CD99 and synaptophysin by immunohistochemistry, and there was focal HMB-45 and microphthalmia transcription factor (MiTF) positivity as well. Fluorescence in situ hybridization confirmed the presence of the t(12;22) (ESWR1-ATF1) translocation.

Spindle Cell Carcinomas of the Upper Aerodigestive Tract

A 65-year-old male with a two-month history of cough and hoarseness underwent direct laryngoscopy which showed a 1.5 cm diameter polypoid glottic mass. A polypectomy was performed revealing spindle cell carcinoma.&#x0D; &#x0D; The World Health Organization (2005) defines a spindle cell carcinoma as “a biphasic tumor composed of a squamous cell carcinoma, either in-situ and/or invasive, and a malignant spindle cell component with a mesenchymal appearance, but of epithelial origin.”1 Spindle cell carcinomas go by a variety of synonyms such as sarcomatoid carcinoma, spindle cell squamous carcinoma and carcinosarcoma.&#x0D; &#x0D; The larynx is a preferred site of involvement where they often present as polypoid masses.1,3 Microscopic examination often shows predominance of the sarcomatoid, spindle-cell component, which can range from fairly bland, reactive-looking fibroblastic-proliferation-like processes, to cytologically malignant and mitotically active proliferations that mimic other spindle-cell sarcomas such as leiomyosarcoma, fibrosarcoma or malignant fibrous histiocytoma.1,2,3 (Figure 1, double arrows) The squamous cell carcinoma component may be in the form of an overlying carcinoma-in-situ, or of a focal keratinizing invasive squamous cell carcinoma that requires multiple sections to disclose.1,2 (Figure 1, single arrow) Cytokeratin-reactivity in the spindle cells, which may be quite focal as in this case, points to their epithelial derivation.1,2,4 (Figure 2)&#x0D; Favorable prognostic findings include polypoid morphology and, like conventional laryngeal squamous cell carcinomas, a low-stage and a glottic site of origin. Reported 5-year survival rates range from 65 – 95%.1&#x0D;

Atypical fibroxanthoma in a woman and literature review

Atypical fibroxanthoma (AFX) is a superficial variant of pleomorphic malignant fibrous histiocytoma. AFX an rare tumor of skin seen mainly in the head and neck region of elderly patients. Clinically, it can be difficult to differentiate from other tumors without histopathological examination. It may clinically resemble squamous cell carcinoma, malignant melanoma, or pyogenic granuloma. The histologic differential diagnosis primarily includes peripheral giant cell granuloma, spindle cell squamous carcinoma, and spindle cell melanoma. Clinical findings and immunohistochemical studies are often needed to establish the diagnosis. The prognosis is generally excellent, although there are rare cases of metastatic disease. The case was 77-year-old female patient. She had a 1.5 cm red-brown bulging lesion on her left cheek that sometimes can bleed for 4 years. As clinically piyogenic granuloma was considered. Excisional biopsi was performed. There was not any lymphadenopathy in her head neck region. Microscopically, the case was interpreted as AFX. The histologic appearance of this lesion was reminiscent of the peripheral giant cell granuloma and giant cell variant of malignant fibrous histiocytoma, also termed malignant giant cell tumor of soft parts. Particularly skin lesions, clinicopathologic correlation is important. Appropriately as can be much easier to diagnose.