Sarcomatous Features Research Articles

Sarcomatoid Morphology in Pediatric Langerhans Cell Neoplasm Does Not Always Predict Aggressive Clinical Course.

Langerhans cell sarcoma (LCS), a rare malignant neoplasm in the general category of myeloid neoplasms characterized by overtly malignant Langerhans cells (LC) with conspicuous mitotic activity including atypical forms. Although most cases occur in adults, rare examples of LCS have been reported in children with variable clinical outcome. We present 2 childhood cases of Langerhans cell neoplasm with high grade sarcomatous features and OSBPL9::BRAF fusion and BRAF V600E mutation.

Rapidly growing massive abdominal sarcomatoid carcinoma on F18-FDG PET-CT scan

Sarcomatoid carcinoma is a rare type of tumour and most commonly arises in the lungs. However, rarely can it also be found in the abdomen. Sarcomatoid tumours are aggressive with large tumoural volume showing cancerous epithelial cells mixed with sarcomatous (nerve, muscle, fat etc.) features on histopathology. Most of the carcinosarcomas arise in the background of pleomorphic adenoma, originating from a myoepithelial precursor. These tumours are resistant to treatment and rapidly metastasize. We present a unique case of hepatocellular sarcomatoid sarcoma, evaluated through F18-FDG PET/CT. Keywords: Sarcomatoid sarcoma, 18F FDG PET-CT, massive abdominal mass.

NCMP-16. A CASE OF WIDESPREAD EXTRACRANIAL METASTASES FROM A GLIOSARCOMA

Abstract BACKGROUND: Glioblastoma (GBM) with sarcomatous features (gliosarcoma) accounts for 2-8% of all glioblastomas1,2, characterized by a biphasic histopathology pattern consisting of glial and sarcomatous components. While GBMs generally do not metastasize outside the central nervous system (CNS), GS has been associated with extracranial metastases in up to 11% of cases3. Case presentation: A 58-year-old right-handed woman with a GBM presented to our institution for a second opinion. She was initially diagnosed 18 months prior and had received radiotherapy with concurrent temozolomide. Her adjuvant temozolomide was discontinued due to rash, and she started on bevacizumab. Her tumor progressed and she sought additional treatment options. On her initial presentation, she was tachycardic with leukocytosis, prompting admission. A chest CT showed thoracic adenopathy, multiple bilateral pulmonary nodules, and a large left malignant pleural effusion. Bronchoscopy revealed spindle cell sarcomatoid neoplasm raising the possibility of metastasis from a CNS primary tumor. Additionally, the patient endorsed development of subacute radicular pain in her bilateral lower extremities as well as weakness. MRI of total spine revealed multilevel osseous and epidural lesions, infiltrating the paraspinal muscles and thickening of cauda roots with concern for leptomeningeal disease. A subsequent PET-CT showed increased FDG avidity in these lesions. Reevaluation of the initial CNS pathology confirmed the presence of mesenchymal/sarcomatous differentiation, consistent with a diagnosis of GS. As confirmed by autopsy, the patient eventually passed away due to widespread and metastatic disease. Discussion: While GBM rarely metastasizes outside the CNS, GBM with sarcomatous features are more likely to exhibit extracranial metastasis. Our patient had widespread involvement, including the lungs, heart, liver, omentum, and bone. Metastasis was thought most likely once EBUS of the lung returned with sarcomatous features. Clinicians should therefore maintain a high suspicion for metastases when dealing with patients diagnosed with GS.

A fortunate presentation of intestinal obstruction secondary to a rare sarcomatoid tumour of the small bowel

Intussusception in the adult is rarely from a benign cause and is almost always pathological. Causes include carcinomas, polyps, Meckel's diverticulum, or colonic diverticulum. Common symptoms include abdominal pain, intestinal obstruction, palpable abdominal mass, gastrointestinal (GI) bleeding, and anaemia. Sarcomatoid carcinoma is a rare type of small intestinal malignancy exhibiting carcinomatous and sarcomatous features. It primarily affects older patients, mean age 57, and is 1.5 times more prevalent in men. This is an interesting case report of a patient presenting with intussusception secondary to a sarcomatoid tumour of the small bowel. Surgery is the treatment of choice in adults with intussusception due to the high malignancy potential. Furthermore, surgical resection of the affected bowel is the definitive form of therapy as small bowel sarcomatoid tumours are not responsive to chemotherapy and radiotherapy. Early surgical intervention helps reduce mortality as it allows for early staging, treatment, and monitoring of the tumour. The patient was fortunate to have presented with intussusception, facilitating early surgical intervention, and was found to have a low disease stage.

Lymph Node and Distant Metastases in Phyllodes Tumor of the Breast.

A 54-year-old woman who incidentally noticed a "knot" in her left breast subsequently underwent excisional biopsy which yielded a diagnosis of malignant neoplasm with sarcomatous features. Given the broad differential diagnosis and imaging findings, the patient underwent bilateral mastectomy and intraoperative sentinel lymph node assessment. An 8.5 cm tumor that was further classified as a malignant phyllodes tumor was identified in the left breast, while the frozen section interpretation came back as positive for metastatic disease which resulted in left axillary lymphadenectomy. Two months later the patient progressed to distant metastatic disease and unfortunately passed away within 6 months after her initial diagnosis. Lymph node metastasis in phyllodes tumor is an exceptionally rare event for which patients usually do not undergo intraoperative sentinel lymph node examination; however high clinical suspicion of metastatic disease would be evidence for further investigation.

Clinical and survival characteristics of primary and secondary gliosarcoma patients

PurposeGliosarcoma (GS) is classified by the World Health Organization as a subtype of glioblastoma with sarcomatous features. GS have a propensity to metastasize, as opposed to other gliomas, with lower 5-year survival rates than GBM patients. In this study, we identified differences in survival between patients with primary and secondary GS. MethodsWe retrospectively identified patients who presented at the MD Anderson Cancer Center with a pathology-confirmed diagnosis of GS. We defined overall survival (OS) from the date of pathological diagnosis of primary GS (from sarcomatous change for secondary GS). We defined progression-free survival (PFS) from the date of GS chemoradiation completion to radiographic disease progression. We used Kaplan-Meier survival estimates and the log-rank test to compare OS and PFS between primary and secondary GS. We used univariable Cox proportional hazard regression to assess differences in OS & PFS by various characteristics. ResultsWe identified 94 GS patients; 70 had primary disease and 24 secondary. Molecular analysis of GS tumor samples revealed that 47.1% were GFAP positive, 38.5% S-100 positive, and 83.7% reticulin-positive. Among the tested samples, 3.8% had IDH and 73.1% had TP53 mutations. The median OS for all patients was 16.8 months. Median OS from the pathological diagnosis of GS was 17.3 months for primary and 10.2 months for secondary GS. Median OS for secondary GS was 28.9 months from initial diagnosis of the primary neoplasm. ConclusionsOur study is the largest single institution evaluation of GS and provides insight into patterns of survival for GS.

Sarcomatoid Yolk Sac Tumor Harbors Somatic Mutations That Are Otherwise Rare in Testicular Germ Cell Tumors.

In testicular germ cell tumors (TGCTs), components with nonspecific sarcomatous features that express keratins and glypican 3 are classified as sarcomatoid yolk sac tumor (SYST). SYST is most frequently seen in metastatic sites after chemotherapy. Like so-called "somatic-type" malignancies arising in TGCTs, SYST is markedly resistant to systemic therapy and has a more aggressive clinical course than conventional types of TGCT. However, the clinicopathologic and molecular features of SYST remain incompletely described. This study evaluated a multi-institutional series of 20 SYSTs using massively parallel sequencing and p53 immunohistochemistry. The histologic and clinical characteristics of the cases were also assessed, including analyses of disease-specific outcomes. DNA sequencing identified somatic mutations in 12/20 cases (60%), including recurrent TP53 and RIF1 mutations (present in 4/20 cases, 20% each). In 3 of the 4 SYST with TP53 mutations, there was molecular evidence of loss of heterozygosity. Immunohistochemistry demonstrated diffuse overexpression of p53 protein in 3/4 (75%) cases with TP53 mutations. The remaining TP53-mutant case demonstrated multifocal overexpression of p53, suggestive of subclonal inactivation of the gene. Overexpression of p53 protein was not seen in any of 15 TP53 wild-type cases evaluated by immunohistochemistry. A subset of 4 cases underwent RNA sequencing (fusion panel), which demonstrated the absence of oncogenic gene fusions. A 2-tiered grading system based on 3 histologic parameters (cellularity, number of mitoses, and necrosis) demonstrated that high-grade SYSTs have a higher risk of disease-specific death compared to low-grade tumors. The risk of disease-specific mortality was also higher in SYSTs with somatic mutations. In conclusion, this study demonstrated that 60% of SYSTs harbor somatic oncogenic mutations that are otherwise rare in TGCTs, and the presence of these mutations is associated with an aggressive clinical course. In addition, the results presented herein suggest that grading SYSTs may be clinically relevant.

Uterine tumor resembling ovarian sex cord tumor: we can diagnose it if we consider it. Case report and literature review

Uterine tumor resembling ovarian sex cord tumor (UTROSCT) represents a rare uterine neoplasm with un­certain malignant potential. Several reports have described re­cur­rences, me­tas­ta­ses and even deaths associated with these generally considered benign tumors, thus making them a controversial disease entity. UTROSCT has an un­clear histogenesis and it possesses a polyphenotypic im­mu­no­phe­no­type. We present a case of UTROSCT in a patient accusing ab­nor­mal genital bleeding and symptoms associated with anemia. After per­for­ming a total abdominal hysterectomy with bilateral sal­pin­gec­tomy, the final pathology report revealed UTROSCT with no sarcomatous features. The pa­tient is still disease-free after 30 months of follow-up. We also present the data avai­la­ble in literature regarding this relative new type of uterine tu­mor. We are of the opinion that a high index of suspicion is of paramount importance when examining a patient with an uterine mass in order to promptly identify the risk factors that may predict agressive tumor behaviour.

Oligosarcomas, IDH-mutant are distinct and aggressive

Oligodendrogliomas are defined at the molecular level by the presence of an IDH mutation and codeletion of chromosomal arms 1p and 19q. In the past, case reports and small studies described gliomas with sarcomatous features arising from oligodendrogliomas, so called oligosarcomas. Here, we report a series of 24 IDH-mutant oligosarcomas from 23 patients forming a distinct methylation class. The tumors were recurrences from prior oligodendrogliomas or developed de novo. Precursor tumors of 12 oligosarcomas were histologically and molecularly indistinguishable from conventional oligodendrogliomas. Oligosarcoma tumor cells were embedded in a dense network of reticulin fibers, frequently showing p53 accumulation, positivity for SMA and CALD1, loss of OLIG2 and gain of H3K27 trimethylation (H3K27me3) as compared to primary lesions. In 5 oligosarcomas no 1p/19q codeletion was detectable, although it was present in the primary lesions. Copy number neutral LOH was determined as underlying mechanism. Oligosarcomas harbored an increased chromosomal copy number variation load with frequent CDKN2A/B deletions. Proteomic profiling demonstrated oligosarcomas to be highly distinct from conventional CNS WHO grade 3 oligodendrogliomas with consistent evidence for a smooth muscle differentiation. Expression of several tumor suppressors was reduced with NF1 being lost frequently. In contrast, oncogenic YAP1 was aberrantly overexpressed in oligosarcomas. Panel sequencing revealed mutations in NF1 and TP53 along with IDH1/2 and TERT promoter mutations. Survival of patients was significantly poorer for oligosarcomas as first recurrence than for grade 3 oligodendrogliomas as first recurrence. These results establish oligosarcomas as a distinct group of IDH-mutant gliomas differing from conventional oligodendrogliomas on the histologic, epigenetic, proteomic, molecular and clinical level. The diagnosis can be based on the combined presence of (a) sarcomatous histology, (b) IDH-mutation and (c) TERT promoter mutation and/or 1p/19q codeletion, or, in unresolved cases, on its characteristic DNA methylation profile.

PATH-23. OLIGOSARCOMA, IDH-MUTANT IS A DISTINCT AGGRESSIVE TYPE

Abstract Oligodendrogliomas are defined by IDH-mutations and the co-deletion of chromosomal arms 1p and 19q. In the past, case reports and small series described gliomas with sarcomatous features arising from oligodendrogliomas, so called oligosarcomas. Here, we report a group of 23 IDH-mutant oligosarcomas that form a distinct methylation group. The tumors were recurrences from prior oligodendrogliomas or developed de novo. Precursor tumors of 11 oligosarcomas were histologically and molecularly indistinguishable from conventional oligodendrogliomas. Tumor cells were embedded in a reticulin fibers network in all oligosarcomas, frequently showing p53 accumulation, positivity for SMA, and regain of H3K27me3 as compared to primary lesions. In 5 oligosarcomas no 1p/19q co-deletion was detectable, even though it was present in the primary lesions. Oligosarcomas harbored an increased chromosomal copy number variation load with frequent CDKN2A/B deletions. Proteomic profiling demonstrated oligosarcomas to be highly distinct from conventional grade 3 oligodendrogliomas with consistent evidence for smooth muscle differentiation. Expression of several tumor suppressors including NF1 and STAG2 was lost whereas oncogenic YAP1 was aberrantly overexpressed in oligosarcomas. Panel sequencing revealed mutations in TP53 and NF1 along with IDH1/2 and TERT promoter mutations. Survival of patients was significantly poorer for oligosarcomas as for grade 3 oligodendrogliomas but comparable to grade 4 IDH-mutant astrocytomas. These results establish oligosarcoma as a distinct type of IDH-mutant glioma differing from conventional oligodendrogliomas on the histologic, epigenetic, proteomic, molecular and clinical level. Diagnosis can be based on the characteristic DNA methylation profile or the combined evidence of sarcomatous histology, IDH-mutation and TERT promoter mutation with or without 1p/19q co-deletion.

Atypical, Extremely Rapid Growth of Pediatric Orbital Neurilemmoma.

Solitary orbital neurilemmoma-a benign tumor of Schwann cells in a peripheral nerve-sheath-are typically very slow growing and present in middle age; in the absence of neurofibromatosis, they are almost never seen in childhood. We describe the clinical presentation, imaging, pathology, and management of this tumor in a 12 years old-the tumor growing extremely rapidly over 5 months and without any evidence of cystic degeneration, hemorrhage, or sarcomatous features. The possibility of tumor growth having been accelerated by prior biopsy is discussed.

Pleural dedifferentiated liposarcoma: A case report.

The present case report describes a rare case of pleural liposarcoma. A 45-year-old Japanese man was hospitalized for increasing left chest pain. Imaging revealed a 10-cm pleural tumor and a 1.7-cm contralateral right pulmonary nodule. Biopsy specimens of the pleural tumor showed undifferentiated spindle-shaped and/or rounded sarcomatous features with myxoid stroma. The patient underwent embolization of the arteries feeding the left pleural tumor and palliative partial resection of the pleural tumor. The surgically removed specimens exhibited similar undifferentiated sarcomatous features. The left pleural tumor regrew aggressively, and the patient succumbed to mortality ~4.2 months following hospitalization. Autopsy demonstrated a 35-cm left pleural tumor, metastasizing to both adrenal glands and lumbar vertebral bones, and a 2.2-cm primary adenocarcinoma of the right lung. The majority of the left pleural tumor and its metastases consisted of undifferentiated sarcomatous elements, however, scattered or aggregated lipoblasts were identified in localized areas adjacent to the diaphragm. Immunohistochemically, these lipoblasts were diffusely positive for MDM2 and focally positive for S-100 protein. Undifferentiated sarcomatous tumor cells were focally positive for MDM2 but negative for S-100 protein. This case was diagnosed as pleural dedifferentiated liposarcoma. The local aggressiveness of the pleural liposarcoma directly contributed to the patient's mortality. A review of the literature indicated that the dedifferentiated subtype may serve as a factor that is indicative of a poor prognosis for pleural liposarcoma.

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

Expression of CD70 (CD27L) Is Associated With Epithelioid and Sarcomatous Features in IDH-Wild-Type Glioblastoma.

Glioblastoma is an aggressive, often recalcitrant disease. In the majority of cases, prognosis is dismal and current therapies only moderately prolong survival. Immunotherapy is increasingly being recognized as an effective treatment modality. CD70 is a transmembrane protein that shows restricted expression in tissue but has been described in various malignancies. Therapeutic targeting of CD70 has demonstrated antitumor efficacy and is in clinical trials. Here, we sought to characterize CD70 expression in a large cohort of gliomas (n = 205) using tissue microarrays. We identified a subset of tumors (n = 18, 8.8% of high-grade gliomas) exhibiting moderate-to-strong immunoreactivity that enriched for the IDH-wild-type glioblastoma variants gliosarcoma (n = 10) and the newly described epithelioid glioblastoma (n = 4). CD70 expression was associated with prolonged survival in gliosarcoma. Analysis of TCGA datasets showed significantly increased CD70 expression in mesenchymal tumors and prolonged survival in recurrent non-G-CIMP high-expressing tumors. In CD70+ gliomas, there was a significant increase in CD68/CD163/HLA-DR+ tumor-associated macrophages, but not CD27+ TIL. These results confirm prior in vitro studies and demonstrate expression in a clinical cohort. The absence of CD70 expression in the post-treatment setting may portend more clinically aggressive disease in gliosarcoma. However, larger-scale studies will be needed to characterize and validate this relationship.

Fibrous hamartoma of infancy: a clinicopathologic study of 145 cases, including 2 with sarcomatous features

Fibrous hamartoma of infancy is a rare soft tissue lesion of infants and young children with characteristic triphasic morphology, which typically occurs in the axilla and less commonly in other locations. We reviewed 145 cases of fibrous hamartoma of infancy from our consultation archives. Cases occurred in 106 males and 39 females (mean age—15 months; range—birth to 14 years), and involved both typical sites (eg, axilla/back/upper arm) (n=69) and unusual locations (n=76). Six were congenital. The tumors presented as subcutaneous masses and ranged from 0.4 to 17 cm (mean 3 cm). All displayed triphasic morphology, but varied widely in the relative percentages of fat, fibroblastic fascicles, and primitive mesenchyme. Hyalinized zones with cracking artifact, mimicking giant cell fibroblastoma, were present in a 44 (30%) of cases; however FISH for PDGFB gene rearrangement was negative in five tested cases. In addition to classical fibrous hamartoma of infancy, two lesions contained large sarcomatous-appearing foci with high cellularity, high nuclear grade, and brisk mitotic activity. One occurred in a 10-month-old female as a new mass in a congenital fibrous hamartoma of infancy; the other occurred as a leg mass in a 6-year-old male. ETV6 gene rearrangement was negative in the tumor from the 10-month-old female. Genomic microarray (OncoScan) showed normal molecular karyotype in eight tested cases, whereas the two tumors with sarcomatous features showed a hyperdiploid/near tetraploid molecular karyotype with copy neutral loss of heterozygosity of chromosomes 1p and 11p, and loss of 10p, chromosome 14, and a large portion of chromosome 22q (22q11.23q13.33), respectively. Follow-up (52 patients; range: 1–208 months, median: 8 months) showed only two local recurrences and no metastases. Extensive local disease in the 10-month-old female with sarcomatous-appearing fibrous hamartoma of infancy necessitated forequarter amputation. In summary, our study confirms the classic clinicopathologic features, including the triphasic morphologic appearance of most cases. In contrast to earlier studies, our series illustrates a broader histologic spectrum than previously appreciated, including its close resemblance to giant cell fibroblastoma in one quarter of cases and the rare presence of ‘sarcomatous' areas, the latter providing evidence that these are complex neoplasms rather than hamartomas.

Vulvar Carcinosarcoma Secondary to Radiotherapy

Carcinosarcomas, a malignancy consisting of squamous cell carcinoma with sarcomatous features, are extremely rare and aggressive tumor of the vulva. Including this case, there are 17 cases reported in the literature. Risk factors for this entity are poorly understood. We describe the case of a rapidly growing primary vulvar carcinosarcoma developing in an 84-year-old woman. The patient had previously received pelvic radiation for a squamous carcinoma of the anal canal. The excised vulvar tumor showed a superficial squamous carcinomatous element, associated vulvar intraepithelial neoplasia, and a transition into deeper sarcomatous component. By immunohistochemistry, the carcinomatous component was positive for keratins and negative for vimentin and smooth muscle actin, whereas the sarcomatous component was negative for keratins and positive for vimentin and smooth muscle actin. The patient was treated with hemivulvectomy with sentinel lymph node dissection followed by limited postoperative chemotherapy. The FIGO stage of the vulvar cancer was stage IB (T1 N0 M0), but even with this low stage, the patient had recurrence 17 months after treatment and died of her disease 8 months later. We compared age and stage at presentation, treatment, disease-free survival, and overall survival of our case to other reported vulvar carcinosarcomas. Vulvar carcinosarcomas are poorly characterized aggressive tumors with poor outcome. This is the first case reported that points to previous radiation exposure as a possible etiologic agent for this lesion.

Merkel Cell Carcinoma (Primary Neuroendocrine Carcinoma of Skin) Mimicking Basal Cell Carcinoma With Review of Different Histopathologic Features

Merkel cell carcinoma (MCC) is a rare but highly aggressive malignancy, which often has typical histopathologic and immunohistochemical (IHC) features. Sometimes the diagnosis is missed because of atypical histological or aberrant IHC findings. A case of MCC that showed irregular lobules of basaloid cells with keratotic areas on the initial shave biopsy is being reported. IHC showed positive staining for high-molecular weight cytokeratin but negative staining for cytokeratin 20, findings consistent with basal cell carcinoma. Subsequent excision specimen showed histopathologic features more typical of MCC. IHC still was negative for cytokeratin 20 but positive for synaptophysin. Review of the literature shows other examples of MCC with basal cell carcinoma-like features. Various other histopathologic differentiations of MCC include those that demonstrate squamous cell and eccrine carcinoma features and those that show melanocytic, lymphomatous, sarcomatous, muscular, and atypical fibroxanthoma-like features. Different histopathologic patterns and mimics of MCC are reviewed to help prevent dermatopathologists from misdiagnosing this aggressive tumor.

A Rare Breast Cancer: Metaplastic Carcinoma

Metaplastic breast cancer (MCB) is a rare tumor with poor prognosis which shows pathologic carcinomatous and sarcomatous features. There are nospecific radiological findings. Radiological findings may vary depending on the components that the lesion contains. We presented a case of metaplastic breast cancer in a 30 year old woman with left cystic lesion who was followed up with intermittant cyst puncture at a different center, and discussed the findings along with the similar cases published in the literature.

Spindle-cell carcinoma of the hypopharynx presenting with a pedunculated appearance: CT and MR features

Spindle-cell carcinoma (SPCC) is a rare tumor that presents biphasic components: squamous-cell carcinoma and sarcomatous features without any differentiation. We present a case of SPCC of the hypopharynx in a 60-year-old man. A well-demarcated mass occupying the hypomesopharynx and presenting a pedunculated appearance was observed originating from the lateral wall of left piriform sinus. High signal intensity on T2-weighted magnetic resonance (MR) images corresponded to the myxoid matrix of the tumor on MR pathologic correlation.

Thymic adenocarcinoma with sarcomatoid features characterized by intracaval tumor growth: Report of a case

Primary thymic adenocarcinoma is an extremely rare tumor. This report describes a case of thymic adenocarcinoma with a peculiar pathological feature and clinical course. A 54-year-old man complained of cough, dyspnea, and chest pain. Computed tomography scans revealed an anterior mediastinal mass with intracaval growth into the superior vena cava. No definitive diagnosis could be made after several examinations and palliative radiotherapy was performed. He passed away 56 days after the appearance of the initial symptoms. An autopsy revealed that the mediastinal tumor obstructed not only the superior vena cava but also the pulmonary arteries. The histological findings revealed the tumor to be an adenocarcinoma with sarcomatous features. The tumor was immunohistochemically positive for CD5 and negative for thyroid transcription factor-1. These results suggested the tumor to be a thymic adenocarcinoma with sarcomatoid features; furthermore, the tumor invaded the great vessels, thus resulting in a poor prognosis.