Atypical Spindle-shaped Cells Research Articles

Malignant transformation of vestibular schwannoma 29 years after Gamma Knife radiosurgery: illustrative case.

Vestibular schwannomas are common posterior fossa tumors, often managed with stereotactic radiosurgery (SRS) due to its low invasiveness and high tumor control rates. However, malignant transformation of vestibular schwannoma (MTVS) is a rare but serious complication with a poor prognosis. The authors present a case of MTVS occurring 29 years after initial SRS, the longest latency reported. A 68-year-old woman developed sudden facial nerve paralysis, with magnetic resonance imaging revealing tumor regrowth. Surgical removal and histopathological analysis confirmed malignant transformation, characterized by spindle-shaped atypical cells and significant p53 positivity. Despite multiple surgeries and radiation therapy, the tumor exhibited rapid regrowth and cerebrospinal dissemination, leading to a poor prognosis. This case underscores the critical importance of long-term monitoring after SRS, as malignant transformation can occur decades later. Additionally, it highlights the potential necessity for early intervention during malignant transformation to improve patient outcomes, despite the challenges in identifying effective treatments for MTVS. https://thejns.org/doi/10.3171/CASE24526.

Retroperitoneal liposarcoma with pancreatic metastasis and gastric cancer: a case report.

On computed tomography scanning, a 63-year-old man with vomiting and anorexia was discovered to have a mass in the pancreatic body and a retroperitoneal mass extending to the right lobe of the liver. An esophagogastroduodenoscopy revealed an advanced gastric carcinoma in the middle gastric body, and a biopsy specimen revealed a poorly differentiated adenocarcinoma. The pancreatic and retroperitoneal masses were considered metastatic lesions of gastric cancer, and a biopsy was taken from the pancreatic lesion using endoscopic ultrasound-guided fine-needle aspiration (EUS-FNA). The histology of the EUS-FNA pancreatic specimen revealed atypical spindle-shaped cells and increased stromal collagen fibrosis, and liposarcoma was considered. Conversely, a percutaneous ultrasound-guided biopsy was taken for the retroperitoneal lesion, and the histology revealed that it was a dedifferentiated liposarcoma. On the basis of histopathological and imaging findings, the retroperitoneal liposarcoma was identified as the primary lesion, the pancreatic lesion as a metastasis of the primary liposarcoma, and the gastric carcinoma as an independent tumor. As far as we know, there have only been three reports of metastatic pancreatic liposarcoma diagnosed via EUS-FNA. In this case, the patient also had gastric cancer, and EUS-FNA was helpful in differentiating metastatic pancreatic tumors from gastric cancer.

Leiomyosarcoma of the upper respiratory tract: A case report and review of literature

Introduction. Leiomyosarcoma is a malignant smooth muscle tumor that is most often found in the gastrointestinal tract, uterus, and retroperitoneum. It is uncommon in the upper respiratory tract. Case report. A 53-year-old woman sought treatment for a 3-month history of nasal obstruction and facial pain. An endoscopic examination revealed a polypoid mass involving the right nasal cavity. The patient was operated on. During surgery, a bulky tumor mass was found, which involved the posterior part of the right inferior concha projecting into the lumen of the epipharynx and upper part of the oropharynx. The lesion was completely excised with adequate margins of uninvolved tissue. Histologic slides stained with hematoxylin and eosin showed interlacing fascicles of atypical spindle-shaped cells. Mitotic figures and bizarre giant cells were frequently observed. Immunohistochemical staining revealed that the tumor was positive for smooth muscle actin and h-caldesmon and negative for S-100. Histological and immunohistochemical features were consistent with leiomyosarcoma. The follow-up was regularly performed by nasal endoscopy and computed tomography, and the disease-free period has been 53 months so far. Conclusion. To the best of our knowledge, this is the longest disease-free period in a patient with leiomyosarcoma of the upper respiratory tract since 1996. This tumor has obscure clinical behavior and prognosis, thus we believe it is necessary to have more published data to determine the best combination of existing therapies, as well as potential new therapies, and enable a longer survival period.

“Fat-Rich” (Spindle Cell-Poor) Variant of Atypical Spindle Cell/Pleomorphic Lipomatous Tumor: Striking Mimic of “Classical” Atypical Lipomatous Tumor/Well-Differentiated Liposarcoma

We report the case of a "fat-rich" (spindle cell-poor) variant of an atypical spindle cell/pleomorphic lipomatous tumor in a 63-year-old female patient presenting with a firm, painless soft tissue mass on the right hip. Atypical spindle cell/pleomorphic lipomatous tumor is a very recently described low-grade adipocytic neoplasm, which occurs predominantly in adults with a predilection for the limbs and limb girdles. In the present case, the diagnosis of an atypical spindle cell/pleomorphic lipomatous tumor was challenging because the tumor was almost exclusively composed of an atypical adipocytic component (resembling "classical" atypical lipomatous tumor/well-differentiated tumor) with only very focal presence of the diagnostic atypical morphologic features (atypical spindle-shaped cells, floret-like multinucleated cells, and "bizarre" pleomorphic [multinucleated] cells). The possibility of a "classical" atypical lipomatous tumor/well-differentiated liposarcoma was ruled out by immunohistochemistry (lack of MDM2 expression and loss of Rb expression) and molecular genetic testing (no amplification of MDM2 and presence of monoallelic deletion of RB1). Another interesting morphologic observation in this case was the striking perivascular location of the atypical spindle/pleomorphic cells in some areas (so-called "pericytic mimicry"). To our knowledge, pericytic mimicry has not been reported in the setting of an atypical spindle cell/pleomorphic lipomatous tumor.

A Case of Spindle Cell Carcinoma of the Larynx

We report herein on a rare case of spindle cell carcinoma in the laryngeal arytenoids. A 60-year-old man presented at our hospital with dyspnea. We performed an emergency tracheostomy to maintain his airway. A biopsy led to the diagnosis of a low specialization type of malignant tumor such as a sarcoma. We performed a total laryngectomy. Postoperative pathologic examination revealed that the greater part of this tumor was composed of spindle-shaped atypical cells and squamous cell carcinoma at an advanced stage, pT3N0M0, so that the final diagnosis was spindle cell carcinoma. We performed postoperative therapy, radiation therapy and chemotherapy with doxorubicin and ifosfamide. Postoperatively the patient has been doing well for 18 months without any sign of recurrence or metastasis. There is as yet no established chemotherapy regimen, so we need to share our experiences regarding chemotherapy for spindle cell carcinomas.

Low-grade myofibroblastic sarcoma of the distal femur

INTRODUCTIONLow-grade myofibroblastic sarcoma (myofibrosarcoma) is described to be a distinct atypical myofibroblastic tumor often with fibromatosis-like features and predilection for head and neck. Low-grade myofibroblastic sarcoma of bone is extremely rare. PRESENTATION OF CASEA 50-year-old woman was admitted to our hospital because she had experienced right knee pain for 2 years. Plain radiography showed a honeycombed lesion on the right distal femur, and computed tomography showed a bone tumor with cortex destruction invading the soft tissue. A biopsy specimen from the intraosseous lesion showed a hypocellular area of spindle cell proliferation with dense collagen deposition, which is reminiscent of a histological feature of desmoplastic fibroma. However, histological examination of the extraosseous lesion indicated a slightly hypercellular area containing scattered spindle-shaped atypical cells with enlarged nuclei, suggestive of low-grade sarcoma. Spindle-shaped atypical cells were immunohistochemically positive for SMA. A final diagnosis of low-grade myofibroiblastic sarcoma of the bone was made from a surgically resected specimen. DISCUSSIONThe patient was alive and well with no evidence of disease at 15 months after the surgery without any additional therapy. CONCLUSIONExtensive sampling of a tumor may be necessary to determine the true nature of the tumor and to make an accurate diagnosis.

Sarcomatoid variant of urothelial carcinoma: Cytological analysis of three cases

Sarcomatoid variant of urothelial carcinoma (SV-UC) is characterized by the presence of biphasic malignant neoplastic components exhibiting morphological and/or immunohistochemical evidence of epithelial and mesenchymal differentiation. SV-UC is a rare variant of UC and the cytological features of this tumor have not been well described. In the present study, we analyzed the cytological features of a series of SV-UC cases; 6 voided urine specimens from 3 patients with SV-UC were reviewed. Several characteristic cytological features were revealed: i) tumor cells were abundant in a necrotic background and while single tumor cells were predominant, small clusters of cells were occasionally present; ii) tumor cells were large-sized and round to polygonal in shape with ill-defined cell borders; iii) tumor cells had a high nuclear/cytoplasmic ratio and enlarged round to oval nuclei containing coarse chromatin and occasional nucleoli; and iv) spindle-shaped atypical cells were rarely identified (1/6 specimens). The cytological features of i), ii) and iii) are indistinguishable from those of conventional invasive high-grade UC. We hypothesize that these tumor cells originated from the conventional high-grade UC component of SV-UC as this component is usually present in this type of lesion, particularly on the surface of the tumor. Moreover, the sarcomatoid component of SV-UC is usually present in the deeper portion of the tumor and therefore detection of this component in the voided cytological specimen is low. Although cytodiagnosis of SV-UC is extremely difficult, cytodiagnosis of malignancy may prove possible due to the presence of a conventional UC component.

Primary Cutaneous Sarcomatoid Anaplastic Large-Cell Lymphoma

A case of primary cutaneous anaplastic large-cell lymphoma with sarcomatoid histologic features is described. Sarcomatoid anaplastic large-cell lymphoma has previously been reported only ten times in the literature. It is a diagnostic challenge because of sarcomatoid features that include atypical spindle-shaped cells, a storiform architecture, and a mucinous stroma. Clues pointing toward the diagnosis of lymphoma may be very subtle. Awareness of this entity can lead to more accurate recognition of a potentially fatal disease.

Epitheloidzelliges Sarkom der rechten Hand

A 35-year-old man presented with swelling, indurations and nodules on the thumb, wrist and fingers of the right hand. History revealed that the findings were slowly progressive and had been present for at least eight years. Histopathologic analysis of a nodule showed a diffuse infiltrate with atypical spindle-shaped cells and expression of cytokeratin, epithelial membrane antigen (EMA) and CD34; the diagnosis of epithelioid sarcoma (ES) was made. Because of diffuse extension of the tumor, forearm amputation was performed along with axillary dissection and local radiotherapy because of axillary lymph node metastases. ES is a rare subtype of soft tissue sarcoma with a harmless appearance and indolent course over years. ES represents a diagnostic challenge, with consequent delay in diagnosis and adequate treatment. The most important measure in the treatment of ES is early surgical excision with adjuvant radiotherapy if local metastases are present.

Primary renal leiomyosarcoma: A diagnostic challenge

Primary leiomyosarcoma is an extremely rare entity constituting only 0.5–1% of all invasive renal tumors. It is frequently diagnosed on histological examination because it does not have any specific diagnostic features clinically and radiologically. At times, it is difficult to differentiate leiomyosarcoma from the sarcomatoid renal cell carcinoma even in histopathology as both the tumors have spindle-shaped atypical cells. Moreover, some epithelial markers can be present in pure smooth muscle sarcomas, while some smooth muscle markers are positive in carcinomas. Hence, a diagnosis of primary renal leiomyosarcoma should be made with caution. Since the prognosis for a renal sarcoma is particularly poor, differentiation from sarcomatoid renal cell carcinoma is necessary. The diagnostic challenge of one such tumor is discussed.

Inflammatory Myxohyaline Tumor of the Distal Extremities

Inflammatory myxohyaline tumor of the distal extremities is an extremely rare low-grade sarcoma with a tendency to produce local recurrence after surgical excision, but with a low metastatic potential. We present the case of a 49-year-old woman with a slow-growing asymptomatic tumor on the right pretibial region that was initially considered to be a lipoma. Histopathology revealed the presence of a polymorphic inflammatory infiltrate within a myxoid and hyaline matrix. Interspersed between the inflammatory cells were 3 different populations of neoplastic cells: atypical spindle-shaped cells; bizarre epithelioid cells, some of which were multinucleated and resembled the virocytes or Reed-Sternberg cells; and cells with abundant, vacuolated cytoplasm, similar to lipoblasts. These clinical-pathologic findings led to a diagnosis of inflammatory myxohyaline tumor of the distal extremities. Although the tumor was excised with wide surgical margins, local recurrence developed after 3 months and was treated with re-excision and radiotherapy.

Tumor mixohialino inflamatorio de las zonas distales de las extremidades

Inflammatory myxohyaline tumor of the distal extremities is an extremely rare low-grade sarcoma with a tendency to produce local recurrence after surgical excision, but with a low metastatic potential. We present the case of a 49-year-old woman with a slow-growing asymptomatic tumor on the right pretibial region that was initially considered to be a lipoma. Histopathology revealed the presence of a polymorphic inflammatory infiltrate within a myxoid and hyaline matrix. Interspersed between the inflammatory cells were 3 different populations of neoplastic cells: atypical spindle-shaped cells; bizarre epithelioid cells, some of which were multinucleated and resembled the virocytes or Reed-Sternberg cells; and cells with abundant, vacuolated cytoplasm, similar to lipoblasts. These clinical-pathologic findings led to a diagnosis of inflammatory myxohyaline tumor of the distal extremities. Although the tumor was excised with wide surgical margins, local recurrence developed after 3 months and was treated with re-excision and radiotherapy.

Interdigitating dendritic cell sarcoma of the ileum recurred in multiple lymph nodes and duodenum three years after operation without chemotherapy

Neoplasms derived from interdigitating dendritic cell are extremely rare. Here we describe a case of a 47-year-old man with interdigitating dendritic cell sarcoma (IDCS) in the ileum. He was admitted to a hospital due to ileus. The ileal tumor, measuring 2 cm, was detected and resected with regional lymphadenectomy. At that time, a pathologic diagnosis of malignant peripheral nerve sheath tumor was made. The patient, who was not treated with chemotherapy, showed no signs of recurrence. After three years, we detected cervical lymphadenopathy and multiple duodenal masses in the patient in our hospital. Oval to spindle-shaped atypical cells, which resembled ileal tumor cells, infiltrated into the lymph node and duodenum. Immunohistochemical staining of these three lesions revealed positivity of S100 protein and several macrophage-related antigens. Based on the histologic and immunohistochemical analysis, the histopathologic diagnosis of IDCS was confirmed. To our knowledge, five cases of IDCS arising in the intestinal tract have been reported to date, and only one case, treated with both surgery and chemotherapy, led to remission. This is the first case that has a comparatively favorable prognosis without chemotherapy after surgery.

Case of uterine cervical carcinosarcoma

Carcinosarcoma (CS) is a rare neoplasm that is called a mixed epithelial and mesenchymal malignancy. CS of the uterine cervix is much less common than its counterparts in the uterine corpus. A 61-year-old, gravida 2, para 2 woman, who had undergone menopause 16 years prior to the presentation, was diagnosed with CS of the uterine cervix. A semiradical hysterectomy was carried out on the diagnosis of stage Ib1 cervical cancer. The patient underwent whole pelvic 45 Gy radiation as a postoperative additional treatment, but she died from multiple organ failure by metastasis 17 months after the operation. The tumor protruded from the cervix to the vagina and measured 4.5 x 3.0 cm. Histologically, the tumor was characterized as a squamous cell carcinoma and mesenchymal malignancy, represented by osteosarcomatous components. The stroma was largely composed of atypical spindle-shaped cells, which were immunohistochemically demonstrated to be of epithelial origin. Uterine cervical CS is one of the aggressive malignancies, and squamous cell carcinomas are common epithelial counterparts of cervical CS as well as adenocarcinomas.

Primary osteosarcoma of the thoracic spine: report of an unusual elderly patient with autopsy findings

A case report of primary osteosarcoma of the spine in an elderly patient. The histopathological features of osteosarcoma vary widely, often leading to diagnostic difficulties particularly when there is little evidence of osteoid formation. The report describes the difficulty in the diagnosis of osteosarcoma of the thoracic vertebra. Department of Orthopaedics and Rehabilitation Medicine, Fukui University Faculty of Medicine, Fukui, Japan. A 78-year-old man presented with paraparesis and underwent urgent anterior excision of a primary spinal tumor emanating from the T10 vertebra followed by artificial vertebral replacement. The patient eventually died of disseminated disease of vertebral osteosarcoma. Samples from the T10 vertebral tumor showed neoplastic growth of atypical spindle-shaped cells, with foci of storiform-like proliferation. The tissue also demonstrated positive immunohistochemical staining for vimentin and alpha-smooth muscle actin and a tentative diagnosis of leiomyosarcoma was made. However, a metastatic nodule of the chest wall at autopsy showed focal osteoid formation, a finding not seen in the primary tumor. Early detection and accurate diagnosis is important for improving not only patient prognosis but also the quality of life. We should always consider this rare entity, particularly in elderly patients who present with back pain and vertebral collapse.

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.

Myxoid Leiomyosarcoma of the Kidney Accompanying Ipsilateral Ureteral Transitional Cell Carcinoma: A Case Report with Cytological, Immunohistochemical and Ultrastructural Study

The authors report a case of myxoid leiomyosarcoma of the kidney accompanying ipsilateral ureteral transitional cell carcinoma. A 74-year-old male patient complained of turbid urine and macroscopic hematuria. He also complained of left back pain, appetite loss and weight loss. Computed tomography revealed a large mass in the left retroperitoneum. Urine cytology disclosed two types of malignant cells, atypical spindle-shaped cells and transitional cell carcinoma. Left total nephro-ureterectomy was performed. The left kidney was occupied by a 6 x 4 x 4 cm, multinodular and mucinous tumor. A transitional cell carcinoma of the left ureter was also observed. The renal tumor was composed of atypical spindle-shaped cells in the mucinous stroma, which showed positive immunoreactivity for anti-muscle-specific actin and anti-desmin antibodies. The ultrastructural examination revealed intracytoplasmic microfilaments with dense bodies, pinocytotic vesicles and junctional structure. These findings were suggestive of the myogenic feature of the case. Urine cytology revealed a number of sarcoma cells in this case since the sarcoma cells markedly invaded the renal pelvis and were apt to separate individually in myxoid stroma. Simultaneous and ipsilateral double malignancies of the renal sarcoma and ureteral transitional cell carcinoma have never been reported in the literature.