Primary Spindle Cell Tumor Research Articles

Primary spindle cell tumor originating from the liver that was difficult to diagnose

BackgroundIt has been reported that hepatocellular carcinoma (HCC) with spindle cell tumor accounts for 1.8% of all HCCs, but spindle cell tumors that do not show an obvious conventional HCC are extremely rare. In this report, we describe a case of resection of a primary spindle cell tumor of the liver that was difficult to diagnose.Case presentationA 75-year-old man presented with fever and right chest pain. He was suspected of a giant primary diaphragmatic tumor of extrahepatic origin by imaging studies. The preoperative differential diagnosis included benign masses such as myxoid sarcoma and schwannoma, and we planned a diaphragmatic resection. Intraoperatively, however, dissection of the tumor from the liver was not possible, requiring an extended right posterior segmentectomy with combined resection of the diaphragm. The patient had a good postoperative course and 1 year has passed since the surgery with no recurrence. The pathology showed that the mass was located just below the hepatic capsule/parenchymal region and was adherent to the diaphragm, but there was no continuity. The morphology suggested a low-grade mesenchymal tumor such as a solitary fibrous tumor and perivascular epithelioid cell tumor, but immunostaining was negative, making the diagnosis difficult. Although some areas of high proliferative activity were observed, finally, the diagnosis of primary spindle cell tumor of the liver with smooth muscle differentiation was made based on the positive results of muscle markers such as αSMA, desmin, and h-caldesmon.ConclusionsSpindle cell tumor arising from the liver is so rare that preoperative and pathological diagnosis is often difficult to reach. Although further studies are needed to elucidate and better understand this uncommon clinical entity, we consider that complete resection is necessary for the above case, which may contribute to long-term survival.

English

Solitary fibrous tumor (SFT) belongs to a group of mesenchymal tumors, first described as a primary spindle cell tumor of the pleura in 1931. Recently, SFT has been reported in various locations with no relation to serosal surface. We describe two such cases in unusual sites of orbit and nasal cavity. Histopathologically, the tumors had a disorganized or patternless arrangement of spindle cells in a collagenous background and prominent vascular channels of varying sizes. Immunohistochemically both tumours showed CD 34 positivity. The two patients had benign clinical course with limited follow-up. SFT should be included in the differential diagnosis of spindle cell tumors arising in the nasal cavity and orbit.

Use of tissue microarrays and immunohistochemistry to standardize the diagnosis of gastrointestinal stromal tumors

We assessed the concordance among seven general pathologists with respect to histologic diagnosis and interpretation of c-kit proto-oncogene (KIT) and platelet-derived growth factor receptor alpha (PDGFRA) immunostaining of 36 cases of primary spindle-cell tumor, predominantly of the gastrointestinal tract, mesentery, and retroperitoneum, based on review of a tissue microarray (TMA) subjected to immunohistochemistry with antibodies to KIT/CD117, PDGFRA, vimentin, desmin, smooth muscle action, CD34, and S-100 protein. Tumors included 20 molecularly analyzed gastrointestinal stromal tumors (GISTs), 4 leiomyosarcomas, 4 schwannomas, 4 desmoid-type fibromatoses, and 4 solitary fibrous tumors. The mean overall concordance with original diagnosis for each histologic type was 91.1%, with a mean kappa value of 0.91. With respect to PDGFRA immunostaining, the four GISTs with PDGFRA mutation were interpreted as cytoplasm positive, but the 16 GISTs with c-kit mutation were interpreted as weak or positive. These results indicate that the overall concordance with original diagnosis in mesenchymal tumors with the use of immunohistochemical panels is high, despite the use of TMAs. To some extent, PDGFRA immunophenotyping may be useful in GISTs with PDGFRA mutation, but it was not highly reproducible or specific. Therefore, in KIT-negative or weakly positive GISTs, mutation analysis will be required.

Follicular dendritic cell sarcoma of the spleen

Diagnosis of primary spindle cell tumors of the spleen is challenging because of the limited immunologic and cytogenetic characterization of this rare entity. We report a case of primary follicular dendritic cell (FDC) sarcoma of the spleen in a 44-year-old woman. Indications for FDC included positive staining for CD21, Ki-M4P, CD14, and fascin. Expression of both standard FDC markers CD23 and CD35 was detected immunohistochemically using tyramide signal amplification. Cytogenetic analysis revealed multiple clonal chromosomal aberrations involving unbalanced translocations of chromosomes X, 3, 5, 7, 8, 9, and 10, leading to net gains at 3q, 7p, 8q, and 9q and net losses at Xp, 8p, 9p, and 10p. Loss at Xp has been described previously in another tumor with FDC features, suggesting that this aberration might play a common role in this malignancy.

Interobserver Variability in Histologic Recognition, Interpretation of KIT Immunostaining, and Determining MIB-1 Labeling Indices in Gastrointestinal Stromal Tumors and Other Spindle Cell Tumors of the Gastrointestinal Tract

The authors studied the concordance among seven pathologists for the histologic diagnosis, interpretation of KIT immunostaining, and determining MIB-1 labeling indices (LI) in 80 adult patients with primary spindle cell tumors, mainly of the gastrointestinal tract, mesentery, retroperitoneum, and pelvis, based on the review of tissue sections using an immunohistochemical panel of antibodies for KIT/CD117, CD34, desmin, smooth muscle actin (SMA), and S-100 protein. Tumors included 30 gastrointestinal stromal tumors (GISTs), 10 leiomyomas, 10 leiomyosarcomas, 10 schwannomas, 10 solitary fibrous tumors, and 10 desmoid-type fibromatoses. The overall concordance with the original diagnosis of each histologic type was 97.9%, the kappa value ranging from 0.95 to 1.00 (mean 0.97), indicating a perfect agreement. The overall interlaboratory concordance with the original interpretation of KIT immunostaining was 91.3%, the kappa value ranging from 0.77 to 0.90 (mean 0.86). The overall interlaboratory concordance with the original interpretation of KIT immunostaining was 91.9%, the kappa value ranging from 0.72 to 0.93 (mean 0.85). The overall concordance for determining MIB-1 LI was 90% with the original evaluation, and the overall kappa value ranged from 0.62 to 0.86 (mean 0.77). These results indicate that it is possible to reliably diagnose GIST and other spindle cell tumors of the gastrointestinal tract with the use of an immunohistochemical panel of antibodies for KIT, CD34, desmin, SMA, and S-100 protein. Although there is clearly unavoidable inter-observer and interlaboratory variability in the interpretation of KIT immunostained sections and interobserver variability in the determination of MIB-1 LI, the concordance between observes is very acceptable, and in most instances such variability can be eliminated by careful reviewing of the hematoxylin and eosin and immunostained sections.

Morphology and immunohistochemical characteristics of hepatic primary and metastatic malignant spindle cell tumors

To investigate the morphology and immunohistochemical characteristics of hepatic primary and metastatic malignant spindle cell tumors, and to conclude the diagnostic and differential diagnostic criteria for these morphologically similar tumors. Forty-six specimens of hepatic spindle cell tumors. 20 primary tumors (43.4%), including 3 cases of sarcomatoid carcinoma (6.5%), 11 of angiosarcoma (23.9%), 2 of epithelioid hemangioendothelioma (5%), 1 of spindle cell carcinoid (2.2%), and 3 of undifferentiated sarcoma (6.5%). and 26 metastatic malignant tumors (56.5%), including 20 cases of gastrointestinal stromal tumors (GIST, 43.4%), 3 of leiomyosarcoma (6.5%), 2 of malignant peripheral never sheath tumor (4.3%), and 1 of meningeal hemangiopericytoma (2.2%), resected during operation or collected during imaging-mediated liver puncture underwent hematoxylin-eosin staining, SP staining, and EnVision immunohistochemical staining. Either primary or metastatic tumors showed extensive overlapping in histopathologic appearance, and hemangiopericytoma-like structure was the predominant pattern, which could be seen in nearly every kind of hepatic spindle cell tumors. The morphology of GIST was the most complex. Two or three entirely different kinds of structure could be seen in one specimen of GIST, especially in the resected ones. Most stromal tumor cases were CD117 positive, and existed the condition that the primary tumor was positive and the metastatic tumor was negative or vice versa or one part of specimen was positive but other part was negative. Leiomyosarcoma was immunoreactive to smooth muscle specific antigen (SMA), malignant peripheral nerve sheath tumor was immunoreactive to S-100 protein and neurofilament (NF), and both were CD117 negative. Angiosarcoma and epithelioid hemangioendothelioma expressed different immunoreactivity to CD31, CD34 and factor VIII related antigen. Sarcomatoid carcinoma was positive for CK and vimentin. Spindle cell carcinoid was positive for CK and neuroendocrine markers, such as synaptophysin and chromogranin A. Primary angiosarcoma is the most common form of primary spindle cell tumor in liver, and metastatic GIST is predominant in hepatic metastatic spindle cell tumors. A penal of immunohistochemical markers is necessary for the final diagnosis of these tumors because of the complexity and similarity of different tumors of this kind.

Symptomatic Pleural Effusion as a rare manifestation of Primary Spindle Cell Tumor of the Pleura

Symptomatic Pleural Effusion as a rare manifestation of Primary Spindle Cell Tumor of the Pleura

Solitary fibrous tumor of the nasal cavity

Solitary fibrous tumor (SFT) belongs to a group of mesenchymal tumors,1 first described as a primary spindle cell tumor of the pleura in 1931.2 Recently, SFT has been reported in various locations with no relation to serosal surfaces. Sites such as the lung,3 liver,4 thyroid,5 sublingual gland,6 and parapharyngeal space7 have been reported. SFTs of the upper respiratory tract, including the nasal cavity, paranasal sinuses, and nasopharynx, are extremely rare, with only 9 previously reported cases.8‐11 Most of the tumors involved the nasal cavity with or without extension to the nasopharynx or the paranasal sinuses. We report the tenth case of SFT of the nasal cavity with extension to the maxillary and ethmoid sinuses.