Spindle-shaped Tumor Cells Research Articles

Giant superficial angiomyxoma of the vulva: a case report and review of the literature

Superficial angiomyxomas (SAMs) are rare, benign cutaneous tumors frequently involving the subcutis. Only 15 cases of SAM involving the vulva have been reported, ranging from 0.9 to 4 cm in diameter. A 26-year-old woman presented with a 7-year history of a large, pedunculated cutaneous mass on the left labium major, measuring 12.5 x 11 x 10.5 cm and mimicking a soft tissue sarcoma. The mass was relatively well-circumscribed, but unencapsulated and multilobulated. Microscopically, the mass showed a conglomerate of moderately-to-sparsely cellular angiomyxoid lobules. Each lobule consisted of scattered spindle-shaped or stellate tumor cells set in an abundant myxoid stroma. Thin-walled, small-to-medium-sized blood vessels were distributed diffusely throughout the stroma. Scattered stromal neutrophils were also observed. No large vessels or plexiform capillaries were apparent. There was no perivascular accentuation of stromal cells or smooth muscle bundles. The tumor cells constantly expressed vimentin, CD34, CD44 and S-100, but none expressed estrogen receptors (ERs) and progesterone receptors (PRs), desmin or cytokeratin. Together, these findings were diagnostic of a SAM. Giant SAMs of the vulva can mimic aggressive angiomyxomas (AAMs) and angiomyofibroblastomas (AMB), as well as soft tissue sarcomas. Giant SAMs should be included in the differential diagnosis of vulvar soft tissue tumors.

Pleomorphic granular cell astrocytoma in the pineal gland: Case report

Pleomorphic granular cell astrocytoma in the pineal region is exceedingly rare, and its clinicopathological features are distinctive. A 67-year-old woman was admitted with a staggering gait. Magnetic resonance imaging revealed a mass lesion at the pineal gland accompanied by obstructive hydrocephalus. Following surgery, pathological examinations demonstrated a pleomorphic granular cell astrocytoma. The patient has been free from recurrence for 24 months after surgery without adjuvant therapy. The specimen exhibited nuclear and cytoplasmic pleomorphism. The nuclei varied in size, shape and coarseness. Variability was also observed in the eosinophilic granular bodies, Rosenthal fibers and spindle-shaped tumor cells. GFAP, S-100 and vimentin were immunohistochemically positive. Reticulin network was absent between the tumor cells, and granular cells with ballooned cytoplasm showing positive staining for PAS. Pleomorphic granular cell astrocytoma is believed to be a form of astrocytoma originating from the pineal gland. Its clinicopathological features resemble those of pleomorphic xanthoastrocytoma. However, it can be differentiated from the latter by the absence of reticulin fibers, absence of basement membrane between adjacent cells, and presence of large numbers of mitochondria.

Spontaneous Basal Cell Carcinoma of the Submandibular Gland in a Rat

At necropsy, a white nodule (about 5 × 3 mm in size) was observed in the right submandibular gland of a 10-week-old female GALAS rat. Histopathologically, oval to spindle-shaped and pale basophilic tumor cells proliferated closely, and formed variably sized foci. The nodule partially spread into or invaded the surrounding normal tissue, and necrotic foci were recognized in the tumor. Immunohistochemically, the nuclei of the tumor cells showed a diffusely positive reaction for p63, and the cytoplasm showed a diffusely positive reaction for cytokeratin and negative reaction for αSMA, vimentin, desmin and S-100. Many tumor cells were positive for PCNA. Ultrastructurally, the tumor cells contained many tonofilaments in the cytoplasm and a few desmosomes at the intercellular portion. Based on these findings, the tumor was diagnosed as a basal cell carcinoma originating from the duct in the rat submandibular gland.

Low-grade myofibroblastic sarcoma of the oral cavity

Two cases of low-grade myofibroblastic sarcoma (LGMS) are presented: one of lateral tongue, the other of lower buccal vestibule. LGMS represents a distinct atypical myofibroblastic tumor that occurs in several sites, primarily within the head and neck regions. A painless, enlarging mass is the most common clinical presentation, but a definitive diagnosis requires both histopathological and immunohistochemical analyses. Histologically, LGMS commonly presents as a cellular lesion composed of spindle-shaped tumor cells arranged primarily in fascicles with a diffusely infiltrative pattern. Immunohistochemically, LGMS shows positive staining for at least one myogenic marker, such as desmin, and muscle actin.

Imprint cytology of primary cardiac sarcomas: a report of 3 cases

Primary cardiac sarcomas are rare instances and only occasionally documented in the cytologic literature. Usually, the diagnosis of these rare lesions can be made at echocardiography, aspiration biopsy cytology, cardiac biopsy, and open cardiac surgery (intraoperative diagnosis). In this study, cytologic configurations and immunohistochemistry for 3 primary cardiac sarcomas (rhabdomyosarcoma, angiosarcoma, and malignant fibrous histiocytoma) were revealed. In rhabdomyosarcoma (right ventricle), the tumor cells exhibited an anisocytotic spindle-shaped nuclei with hyperchromasia and an obscure cytoplasmic margin. Vimentin and myosin were positive throughout the cytoplasm for the tumor cells. In angiosarcoma (right atrium), small clusters of anisocytotic spindle-shaped tumor cells appeared as vascular-like structures and hemosiderin-laden macrophages in many erythrocyte-rich backgrounds. Nuclei showed round to oval shape with hyperchromasia and prominent large nucleoli. Cytoplasm was obscure and elongated. Factor VIII related antigen and CD34 were strongly positive throughout the cytoplasm for the tumor cells. In malignant fibrous histiocytoma (right ventricle), the tumor cells exhibited oval to spindle-shaped and elongated nuclei and coarse granular chromatins with hyperchromasia. The nuclear margin was thin. A few small round nucleoli appeared. Elongated obscure and foamy cytoplasm was stained pale blue. Vimentin and alpha(1)-antitrypsin were positive throughout the cytoplasm for the tumor cells. This study elucidated the cellular characteristics and immunohistochemistry for cardiac sarcomas using imprint smears as an aid to cytopathologic diagnosis.

Gastrointestinal stromal tumor of the lesser omentum in a young adult patient with a history of hepatoblastoma: Report of a case

A gastrointestinal stromal tumor (GIST) of the lesser omentum is extremely rare. This report presents a case of GIST of the lesser omentum in a 22-year-old man with a history of hepatoblastoma. Computed tomography showed an abdominal mass about 30 mm in diameter adjacent to the lesser wall of the stomach. A laparotomy showed a mass of about 27 x 24 x 20 mm in diameter originating from the lesser omentum but isolated from the stomach and the liver. Histopathology showed that the tumor was composed of spindle-shaped tumor cells with high cellularity. Immunohistochemically, the tumor was positive for KIT and CD34. An in-frame deletion was observed in the c-kit gene exon 11. Therefore, the tumor was diagnosed as a GIST originating from the lesser omentum.

Solitary subependymal giant cell astrocytoma incidentally found at autopsy in an elderly woman without tuberous sclerosis complex

Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the setting of tuberous sclerosis complex (TSC). However there are several reported cases in which patients with a solitary SEGA had no other stigmata of TSC. We describe a case of SEGA in a 75-year-old woman representing the oldest patient reported to-date. The patient had a history of radical vulvectomy for malignant melanoma (MM), and died of autopsy-confirmed widespread systemic metastasis. Postmortem examination of the brain revealed a single 2.1 x 1.0 x 0.8 cm intraventricular nodule in the lateral ventricle. Histologically, it was composed of interlacing bundles of spindle-shaped tumor cells with thin delicate processes admixed with relatively large pleomorphic cells with abundant glassy eosinophilic cytoplasm, as seen in a SEGA. Immunohistochemically, GFAP, S-100 protein, and neuron specific enolase were positive, and synaptophysin labeled a few tumor cells. Also noted were rare isolated MM cells within the tumor (i.e., tumor-to-tumor metastasis). Autopsy showed no manifestations of TSC systemically or intracranially. The histopathological differential diagnosis was limited and included giant cell ependymoma and, much less likely, giant cell glioblastoma and pleomorphic xanthoastrocytoma. This case illustrates that SEGA can be found incidentally in an elderly individual with no associated symptoms and also indicates that SEGA can occur outside the setting of TSC. Tumor metastasis to an occult SEGA is extremely rare.

Malignant glomus tumour in a German shepherd dog

A solitary mass, 2.5 cm x 2.5 cm x 2.0 cm in size, was found in the subcutis adjacent to the ischial tuberosity of an 8-year-old male German shepherd dog. The excised mass was not encapsulated and the cut surface was solid, grey-white and had multiple red areas suggestive of haemorrhage. Microscopically, the neoplasm was composed of epithelioid cells interspersed with venous vessels of various sizes and irregular bundles of spindle-shaped tumour cells with mucinous stroma. The nuclei showed anisokaryosis and many mitotic figures were noted. Immunohistochemically, the majority of tumour cells were positive for alpha smooth-muscle actin and vimentin, but negative for cytokeratin, desmin, S-100 and factor VIII-related antigen. Ultrastructurally, the tumour cells contained irregular nuclei, few mitochondria, few rough endoplasmic reticulum, cytoplasmic myofilaments of intermediate density, pinocytotic vesicles and distinct basal lamina. Based on these pathological findings, the diagnosis was malignant glomus tumour.

Dermatomyofibroma: Clinicopathologic and Immunohistochemical Analysis of 56 Cases and Reappraisal of a Rare and Distinct Cutaneous Neoplasm

Dermatomyofibroma represents a rare and distinct benign cutaneous mesenchymal neoplasm of fibroblastic/myofibroblastic differentiation. A series of 56 cases of dermatomyofibroma has been analyzed to further characterize the clinicopathologic spectrum of this entity. Forty patients were female and 8 were male (gender was unknown in 8 cases). Patients' age ranged from 3 to 51 years (mean 30.8 years, median 30 years). Interestingly, 6 patients were younger than 16 years, and in this age group, 3 male and 3 female patients, respectively, were noted. The shoulder (13 cases) was the anatomic site most commonly affected, followed by the upper arm (7 cases), the neck (6 cases), the thigh (6 cases), the chest wall (4 cases), the back (3 cases), the axillary fold (2 cases), the abdominal wall (2 cases), and 1 case each was seen on the forearm, the buttock, and the popliteal fossa (exact anatomic location was unknown in 10 cases). One patient presented with 2 lesions arising simultaneously on both shoulders. Histologically, an ill-defined, plaque-like dermal neoplasm of varying cellularity was seen in all cases, composed of bland spindle-shaped tumor cells often oriented parallel to the overlying epidermis. An infiltration of superficial part of the subcutis was seen in 23 cases, and in 6 cases, deeper parts of the subcutis were involved by often perpendicular growing bands of neoplastic cells. Immunohistochemically, tumor cells in 11 of 48 cases tested stained positively for alpha-smooth muscle actin, and a focal expression of this marker was noted in 20 cases. In addition, a focal expression of CD34 was seen in 10 of 45 cases tested. Follow-up information was available in 38 cases (range from 3 to 156 months, median 34 months), and despite marginal or incomplete excision in 17 cases, none of the cases recurred. Dermatomyofibroma represents a benign fibroblastic/myofibroblastic dermal neoplasm.

Extraskeletal Osteosarcoma of the Thigh: An Autopsy Case Report

We report a case of extraskeletal osteosarcoma (ESOS) and autopsy findings. A 35-year-old man presented with an ossified tumor in the right thigh and lung metastasis. The lung tumors continued to develop despite multiagent chemotherapy and caused death within 8 months. Autopsy revealed many secondary lesions in the lungs, especially in the left lung. Histopathologically, the primary tumor and one of the secondary tumors showed proliferation of spindle-shaped tumor cells focally forming lace-like osteoid material. Therefore, we made a definite diagnosis of ESOS.

Pleomorphic carcinoma of the lung expressing podoplanin and calretinin

Pleomorphic carcinoma (PC) of the lung is classified as a subtype of sarcomatoid carcinoma of the lung, and peripheral PC is sometimes difficult to differentiate from the sarcomatoid component of mesothelioma. An 80-year-old man was referred to National Cancer Center Hospital East because a chest X-ray showed an abnormal shadow. CT scans of the chest indicated two solid masses located in the right lower lobe, and CT-guided needle biopsy yielded spindle-shaped tumor cells that were immunoreactive for both podoplanin and calretinin. Mesothelioma could not be ruled out, and the tumors were surgically resected to facilitate definitive pathological diagnosis. Both tumors were composed of undifferentiated carcinoma, bronchioloalveolar carcinoma and spindle cell carcinoma, and spindle cell component was immunoreactive for podoplanin and calretinin. Ten other tumors diagnosed as peripheral PC were also tested for podoplanin and calretinin expression. The sarcomatoid component in four of the 11 cases (36%) was immunoreactive with podoplanin, and it was calretinin positive in nine of the 11 cases (82%). When making the differential diagnosis between PC and the sarcomatoid component of mesothelioma, care is required in diagnosing biopsy specimens of peripheral lung spindle-cell tumors that are positive for both podoplanin and calretinin.

Immunohistochemistry and K‐ras sequence of pancreatic carcinosarcoma

Herein is presented a case of carcinosarcoma of the pancreas in an 82-year-old woman, analyzed on immunohistochemistry and K-ras sequence. The tumor, which arose in the pancreas head, was removed on pancreaticoduodenectomy. The patient died, however, of disseminated intravascular coagulation syndrome from postoperative sepsis 13 days later. Microscopically, the tumor consisted of malignant epithelial (well-differentiated adenocarcinoma cells) and mesenchymal (spindle-shaped tumor cells) components. The adenocarcinoma cells had positive immunostaining for cytokeratin AE1/AE3, cytokeratin 7, epithelial membrane antigen (EMA), CEA and carbohydrate antigen 19-9 (CA 19-9), while focal staining of these proteins was observed in the sarcomatous cells. In contrast, the sarcomatous cells had diffuse immunostaining for vimentin, CD10 and p53, while these proteins were not expressed in the ductal adenocarcinoma cells. These findings support the dual characteristics of a carcinosarcoma. DNA sequencing of the present case indicated point mutations of K-ras in both codons 12 and 34 on exon 2. The latter mutation is likely to correlate with the sarcomatous characteristics of this tumor. The tumor cells had specific and diffuse positive staining for CD10 and p53, with features characteristic of rapid growth.

Solitary Fibrous Tumour of the Floor of the Mouth

This report is of an 84-year-old man with a solitary fibrous tumour of the oral cavity. He was diagnosed with a benign tumour, and surgical excision was performed. Microscopic examination revealed haphazardly arranged spindle-shaped tumour cells. Immunohistochemically, the tumour cells were positive for clusters of differentiation34, B-cell lymphoma-2, and vimentin, and negative for epithelial, myogenic, neurogenic and histiocytic markers. To the authors' knowledge, this is only the fourth reported patient with solitary fibrous tumour of the oral cavity involving the oral floor.

Aggressive angiomyxoma of the lung

Aggressive angiomyxoma (AAM) is a rare mesenchymal neoplasm that usually occurs in the pelvic–perineal region. Only two cases of AAM occurring outside this region have been reported. The case of...

Cardiac tumor comprising two components including typical myxoma and atypical hypercellularity suggesting a malignant change

Cardiac myxoma is a benign neoplasm and is not considered to carry a risk of malignant transformation. We describe a case of a cardiac tumor comprising typical myxoma and atypical cellular parts. A 72-year-old woman was found to have a tumor in the left atrial chamber after undergoing echocardiographic investigation and so she underwent tumor excision surgery. The excised tumor was composed of two components with different features with a smooth transition from one type to the other. The apex of the tumor showed a gelatinous and villous appearance on gross examination, and histological examination revealed that the tumor cells had small oval nuclei and eosinophilic cytoplasm and were arranged in cords and vasoformative structures in the myxoid matrix. The base of the tumor appeared as a solid mass on gross examination with hypercellular proliferation of spindle-shaped tumor cells and intersecting fascicles observed histologically. Nuclear mitosis was seen in more than 10/10 high-power fields, coagulation necrosis was prominent, and invasion of the atrial wall was seen. On immunohistochemistry, the tumor cells were found to be positive for alpha-smooth muscle actin, calretinin, and CD34, and this immunoreactivity was decreased at the tip. Ki-67 labeling index was increased at the apex. We diagnosed this tumor as cardiac myxoma with an atypical hypercellular component suggesting a malignant change. It is important to recognize that a cardiac myxoma could be accompanied by atypical change.

Aggressive angiomyxoma of supraclavicular fossa: A case report

Aggressive angiomyxoma (AAM) is a rare myxoid mesenchymal tumor that occurs almost exclusively in the adult pelvic-perineal region and predominantly in females. Only 1 case of AAM occurring outside this region has been reported. Here we report another such case. The patient was referred for evaluation of a firm nonmovable mass of the supraclavicular fossa that had progressively enlarged within the previous year. MRI showed an infiltrative growth pattern with adhesion to adjacent anatomic structures. Wide excision was attempted, but a clear margin could not be achieved. The histopathology revealed characteristic features of AAM, including stellate to spindle-shaped tumor cells set in a myxoid background, with hyalinizing thick-walled vessels and characteristic immunophenotype. Accurate diagnosis and a definite surgical margin are crucial because AAM is locally aggressive and easily recurrent. Our case deserves attention because it shows that AAM may exist in the head and neck.

Subependymal giant cell astrocytoma incidentally found in an elderly patient: Report of an autopsy case

Background: Subependymal giant cell astrocytoma (SEGA) is a benign, slowly growing tumor typically occurring in the first two decades of life. SEGA usually occurs in the setting of tuberous sclerosis complex (TSC), however there are several reported cases in which patients with solitary SEGA had no other stigmata of TSC. These lesions are generally considered to be forme fruste of TSC. We report such a case of SEGA in the oldest patient reported to date.Case report: The patient was a 75‐year‐old woman who had a history of radical vulvectomy for malignant melanoma and died of widespread systemic metastasis, which was confirmed at autopsy. Postmortem examination of the brain revealed a single intraventricular nodule in the left lateral ventricle, measuring 2.1×1.0×0.8cm. Histologically, the tumor was composed of interlacing bundles of spindle shaped tumor cells with thin delicate processes admixed with relatively large atypical cells with abundant glassy eosinophilic cytoplasm, as seen in SEGA. Ultrastructural study supported the diagnosis. Autopsy study showed no manifestations of TSC systemically or intracranially.Discussion: This is a very rare case of solitary SEGA incidentally found at autopsy in an elderly patient lacking any other findings of TSC. This is the second reported case of SEGA found in an elderly patient. This autopsy case showed that SEGA can occur outside the setting of TSC.

Complex t(5;8) involving the CSPG2 and PTK2B genes in a case of dermatofibrosarcoma protuberans without the COL1A1-PDGFB fusion

Dermatofibrosarcoma protuberans (DFSP) is a rare, dermal neoplasm of intermediate malignancy. It is made of spindle-shaped tumor cells in a storiform pattern positive for CD34. Cytogenetically, DFSP cells are characterized by either supernumerary ring chromosomes composed of sequences derived from chromosomes 17 and 22 or more rarely of translocations t(17;22). These chromosomal rearrangements lead to the formation of a specific chimeric gene fusing COL1A1 to PDGFB. So far, the COL1A1-PDGFB fusion gene remains the sole fusion gene identified in DFSP. However, some observations suggest that genes, other than COL1A1 and PDGFB, might be involved in some DFSP cases. We report in this paper a DFSP case presenting as a unique chromosomal abnormality a complex translocation between chromosomes 5 and 8. This is the first report of a DFSP case where the lack of chromosomes 17 and 22 rearrangement and the absence of COL1A1-PDGFB fusion gene have been demonstrated. Using fluorescence in situ hybridization analysis, we showed that the CSPG2 gene at 5q14.3 and the PTK2B gene at 8p21.2 were disrupted by this rearrangement. Although rare, the existence of cases of DFSP negative for the COL1A1-PDGFB fusion has to be taken in consideration when performing molecular diagnosis for a tumor suspected to be a DFSP.

A Spindle Cell Predominant Pancreatic Solid-pseudopapillary Tumor

A hitherto unrecognized variant of solid-pseudopapillary tumor (SPT) of the pancreas is reported. The tumor presented in the pancreatic tail of a 44-year-old female patient. It was a well-defined, solid nodule measuring 25 mm in diameter, with homogenous tan gray cut surface. Histologically, the neoplasm was mostly composed of sheets of spindle cells. No cellular atypia and mitosis was identified. The periphery of the tumor showed typical feature of SPT. Immunohistochemically, the tumor cells were positive for vimentin, CD10, CD56, β-catenin, and α1-antichymotrypsin, but negative for cytokeratin, chromogranin, synaptophysin and S-100 protein. Ultrastructurally, the tumor showed a few acinar spaces with microvilli between tumor cells. This case is peculiar in that the tumor did not show gross cystic change and predominantly consists of spindle shaped tumor cells, so may cause difficult diagnostic problem.

Astroblastoma: A radio-histological diagnosis

Astroblastoma is thought to arise from astroblast - an intermittent cell between spongioblast and astrocytes and is a rare tumor in the pediatric age group. The histological appearance are characterized by radiating arrangement of spindle-shaped tumor cells forming perivascular pseudorosettes, and being very similar to that seen in ependymomas, makes differentiation difficult. The authors report the case of a 5-year-old girl who had a large frontal tumor, which was thought to be an astroblastoma preoperatively, based on the unique magnetic resonance imaging features these tumors have. The histological findings, in association with the preoperative radiology confirmed the diagnosis of astroblastoma. The authors conclude that the MR imaging of astroblastomas is extremely helpful in differentiating from ependymomas and is used for preparing the histopathology reports in these patients.