Sarcomatoid Appearance Research Articles

Sarcomatoid Carcinoma of the Prostate

Sarcomatoid carcinoma of the prostate is among the rarest malignant neoplasm types and has been well known for its aggressive clinical course. Patient was admitted with the symptoms of lower urinary tract. Transurethral resection of prostate (TUR-P) was carried out. Revealing Gleason 5 + 3 = 8 prostate adenocarcinoma in TUR-P material. Thereby, a Radical Prostatectomy procedure was planned. In operation, frozen examination revealed adenocarcinoma metastasis to the obturator lymph node. The operation was terminated. In the postoperative 3rd month, the patient was re-admitted with acute urinary system symptoms. A cystoscopy performed and complete resection of the mass was performed. The pathological examination reported that the tumor was compatible with undifferentiated adenocarcinoma owing to presence of poorly differentiated tumoral cells and detection of adenocarcinoma in a relatively small (<1%) focus. 4 month after the operation, the patient underwent another cyctoscopic examination which revealed the prostatic lounge and most of the bladder lumen to be filled with tumoral tissue. The tumoral tissues was resected incompletely. This material was diagnosed to be “Sarcomatoid Malignant Tumor” upon the new evidences of progressive dedifferentiation and predominant sarcomatoid appearance, compared with the former TUR-P materials. Subsequent PET-CT scan depicted multiple metastasis. The patient was referred to oncology department. In conclusion, sarcomatoid carcinoma is a malignant variant that brings along diagnostic and treatment difficulties.

Myxoid intravascular fasciitis

Nodular fasciitis is a common reactive proliferation of soft tissue. It is composed of high density of fibroblasts and myofibroblasts, and sometimes shows a sarcomatoid appearance histologically. A rare variant of nodular fasciitis, intravascular fasciitis can be found in the veins or arteries. Histologically, intravascular fasciitis has been described that resembles nodular fasciitis with a less prominent mucoid matrix. Here, we report a case of intravascular fasciitis with prominent mucin deposition. Clinically, it presented as multiple nodules on the leg of the patient. Histologically, it showed proliferation of spindle cells in a vein of the dermis and Alcian blue staining confirmed prominent mucin deposition between the spindle cells. Immunohistochemistry studies showed the spindle cells were positive for vimentin, actin and smooth muscle actin, but negative for keratin, S100 protein, desmin, CD31 and CD34. Based on the clinical and histopathological properties, a diagnosis of myxoid intravascular fasciitis was made.

Histiocytic sarcoma: a clinicopathologic study of 6 cases

To study the morphologic features, immunophenotypes, differential diagnoses and prognosis of histiocytic sarcoma (HS). The clinical and pathologic findings of 6 cases of HS were reviewed. Immunohistochemical assay (Elivision staining) was also performed. Follow-up information was available in 4 patients. There were altogether 3 males and 3 females. The age of patients ranged from 12 to 81 years old (median = 54.6 years). The sites of involvement included lymph node (number = 2 cases) and skin or soft tissue (number = 4 cases). The tumor was composed of sheets of large epithelioid cells with abundant eosinophilic cytoplasm, oval to irregular nuclei, vesicular chromatin and large nucleoli. Binucleated form was not uncommon. Two of the cases showed increased pleomorphism with multinucleated tumor giant cell formation. Focal cytoplasmic with foamy appearance was identified in 3 cases. One case demonstrated foci of spindly sarcomatoid appearance. Hemophagocytosis was identified in 2 cases. Mitotic figures were readily identified. The tumor cells were often accompanied by various numbers of inflammatory cells. Immunohistochemical study showed that all cases were diffusely positive for leukocyte common antigen, CD4, CD68 and CD163. Four of the 5 cases studied also expressed lysozyme. Amongst the 4 patients with follow-up information available, 3 died of the disease at 6 to 11 months interval after diagnosis. One patient, whose lesion was localized at the skin and soft tissue, survived for 3 years, with no evidence of tumor recurrence. Accurate diagnosis of the HS is based on the combination of morphologic examination and immunohistochemical assay. HS often presents with clinically advanced disease and pursues an aggressive clinical course, with a poor response to therapy. However, a subset of cases presenting with clinically localized lesion may carry a relatively favorable long-term outcome.

A case of cotyledonoid leiomyoma and review of the literature

We report a case of a rare, unusual benign tumor, the cotyledonoid leiomyoma or Sternberg tumor, detected incidentally in a 67-year-old woman who underwent total hysterectomy and bilateral salpingo-oophorectomy because of a persisting left ovarian cyst of 5 cm. This is the 14th case of cotyledonoid leiomyoma and 2nd case which had no intrauterine portion but had extrauterine extensions. At laparotomy, it was detected that a deep red nodular papillary tumor had been dissecting the posterior leaf of the left broad ligament, projecting into the abdominal cavity just near the left cornu of the uterus, and extending in downward direction. The remaining portion was lying between the layers of the left broad ligament and arose from the subserosa of the left side of the uterine isthmus. Although the bizarre and unusual shape was in favor of malignity, the frozen-section examination revealed a benign histology. Although the cotyledonoid leiomyoma is a benign entity, it may suggest a malignant disease owing to its unusual sarcomatoid appearance and its rarity. Therefore, awareness of obstetricians and gynecologists regarding this rare entity will prevent unnecessary anxiety and interventions.

A case of cotyledonoid leiomyoma and review of the literature

We report a case of a rare, unusual benign tumor, the cotyledonoid leiomyoma or Sternberg tumor, detected incidentally in a 67-year-old woman who underwent total hysterectomy and bilateral salpingo-oophorectomy because of a persisting left ovarian cyst of 5 cm. This is the 14th case of cotyledonoid leiomyoma and 2nd case which had no intrauterine portion but had extrauterine extensions. At laparotomy, it was detected that a deep red nodular papillary tumor had been dissecting the posterior leaf of the left broad ligament, projecting into the abdominal cavity just near the left cornu of the uterus, and extending in downward direction. The remaining portion was lying between the layers of the left broad ligament and arose from the subserosa of the left side of the uterine isthmus. Although the bizarre and unusual shape was in favor of malignity, the frozen-section examination revealed a benign histology. Although the cotyledonoid leiomyoma is a benign entity, it may suggest a malignant disease owing to its unusual sarcomatoid appearance and its rarity. Therefore, awareness of obstetricians and gynecologists regarding this rare entity will prevent unnecessary anxiety and interventions.

Afectación cutánea específica en pacientes con mieloma múltiple. Estudio clínico-patológico, inmunohistoquímico y citogenético de 40 casos

Introduction Specific cutaneous involvement in patients with multiple myeloma is very rare. When it does occur, it is in patients with advanced stages of multiple myeloma and with a large tumorous mass. In this study, we analyzed 40 patients with specific cutaneous involvement from multiple myeloma, with no bony lesions underlying the skin lesions, and we reviewed the related literature. Objectives We were particularly interested in the clinical course of these patients, including survival once skin metastases had developed and the possible influence of the different treatments administered. Material and methods The study was completed by determining the histopathological pattern, the immunohistochemical profile, the type of immunoglobulin and immunoglobulin light chain produced by the neoplastic plasma cells that infiltrated the skin, as well as the cytogenetic characteristics of these cells. Results From a clinical standpoint, the skin lesions consisted of multiple nodules or plaques with erythematous or violaceous coloration, and variable location. Histopathologically, two patterns were seen: nodular and diffuse interstitial. The plasma cells showed cytological atypia, and in one case they displayed a spindle shape, giving the lesion a sarcomatoid appearance. Immunohistochemically, these neoplastic plasma cells were strongly positive for CD79a, CD138, and EMA, while the immunoexpression of CD38 and CD43 was less intense and constant. In 39 of the 40 patients, the type of monoclonal immunoglobulin produced by the neoplastic plasma cells in the skin lesions was determined: 18 patients had IgA myeloma (5 IgA κ and 13 IgA λ), 19 patients had IgG myeloma (15 IgG κ and 4 IgG λ) and 2 patients had IgD λ myeloma. The 22 cases in which immunoglobulin heavy chain gene rearrangement studies by PCR were carried out for the JH gene showed monoclonal rearrangement, while viral studies to try to identify genetic material of the HHV-8 virus and the Epstein- Barr virus gave negative results in all cases. These 22 cases studied using FISH showed the deletion of the rb-1 retinoblastoma gene in the neoplastic plasma cells that infiltrated the dermis. Despite aggressive chemotherapy, all of the patients died a few months after the skin lesions developed. Conclusions In our series, a perfect correlation was seen between the findings of serum electrophoresis and the immunohistochemistry of the skin lesions with regard to the type of immunoglobulin and the immunoglobulin light chain restriction produced by the neoplastic plasma cells. Patients with multiple myeloma have a very short survival period once specific skin lesions appear, regardless of the therapy administered. The deletion of the rb 1 gene may be a prognosis marker to identify those patients with especially aggressive forms of multiple myeloma.

Mesonephric Adenocarcinoma of the Uterine Corpus

Mesonephric (wolffian) neoplasms of the female genital tract are infrequent and found in sites where embryonic remnants of wolffian origin are usually detected, such as the uterine cervix, broad ligament, mesosalpinx, and ovary. Their diagnosis is difficult because of the absence of specific immunohistochemical markers for mesonephric derivatives. We present the first report of adenocarcinoma of mesonephric type arising as a purely myometrial mass without endometrial or cervical involvement in the uterine corpus of a 33-year-old woman. The tumor showed a combination of patterns, with retiform areas, ductal foci, and small tubules with eosinophilic secretion, which merged with solid sheets of cells with a sarcomatoid appearance. Immunohistochemically, neoplastic cells were diffusely positive for cytokeratin 7, epithelial membrane antigen, and CD15 and focally positive for BerEP4 and vimentin. A hitherto unreported feature was the positivity for CD10 in neoplastic cells, which was also present in a large number of control tissues obtained from male mesonephric derivatives and female mesonephric remnants and tumors. Furthermore, CD10 was negative in controls from müllerian epithelia of the female genital tract and in their corresponding tumors. Therefore, the expression of CD10 by mesonephric remnants may be useful in establishing the diagnosis of tumors with mesonephric differentiation.

上顎洞に生じた紡錘細胞癌の１例

Spindle cell carcinoma is considered a subtype of squamous cell carcinoma that has a sarcomatoid appearance. In the head and neck region, cases of spindle cell carcinoma of the larynx have occasionally been reported. However, spindle cell cacrcinoma of the maxillary sinus is extremely rare. We describe a case of spindle cell carcinoma of the maxillary sinus in a 53-year-old woman. Surgical treatment was performed under the clinical diagnosis of a maxillary neoplasm. During the operation, a biopsy specimen was taken, and the histopathological diagnosis was a spindle cell carcinoma. The lesion was removed under general anesthesia. Radiotherapy and chemotherapy were additionally given to prevent recurrence and metastasis of the tumor.There has been no evidence of recurrence as of 5 years 6 months after surgrey.

Malignant aneuploid spindle/cell transformation in a proliferating trichilemmal tumour.

A case of spindle-cell carcinoma developed on a proliferating trichilemmal tumour (PTT) is presented. The tumour on the scalp of an 84-year-old white woman was excised and histological examination showed typical features of PTT, but with a coexistent malignant spindle-cell tumour with a sarcomatoid appearance. There were some areas of transition between squamous trichilemmal epithelium and the neoplastic mass composed of atypical spindle-shaped cells. Two years later, the patient was well, and neither recurrence nor metastases were observed. We report the second case of PTT with a malignant spindle-cell transformation with a sarcomatoid appearance (spindle-cell carcinoma), and a proven aneuploidy, demonstrated with DNA-flow cytometric analysis.

Large-cell hematolymphoid neoplasms of uncertain lineage

In almost every large study attempting to characterize non-Hodgkin's lymphomas, there is a small subset of tumors for which the lineage remains poorly defined. The investigators studied a series of 20 hematolymphoid neoplasms that could not be clearly assigned to the B or T cell lineage by phenotypic criteria. Histologically, 12 cases had an appearance suggesting a histiocytic origin, seven cases resembled a pleomorphic immunoblastic lymphoma, and one had a sarcomatoid appearance. By immunologic studies, a variety of B cell, T cell, and monocyte/macrophage markers were expressed on the neoplasms, often with coexpression of markers for different lineages. Twelve cases expressed the Ki-1 antigen. In immunogenotyping studies of T cell receptor (TCR) and immunoglobulin genes, 13 cases showed clonal rearrangements of the beta or gamma TCR gene; one of these cases also had clonal rearrangements of a light chain immunoglobulin gene. Seven cases showed a germline configuration with all combinations of probes and enzymes used. We conclude that a small subset of hematolymphoid neoplasms shows a pattern of diverse immunologic marker expression that does not appear to reflect normal differentiation. However, a majority of these cases contain clonal TCR gene rearrangements, suggesting a frequent relationship to the T lineage.