Sacrococcygeal chordoma with a malignant spindle cell component. A report of two autopsy cases with a review of the literature.

Two autopsy cases of sacrococcygeal chordoma which showed sarcomatous components in the primary and metastatic tumors are reported. Case 1 was a 48-year-old female who died 9 years after the development of the initial symptoms. Autopsy findings showed metastatic tumors consisted of malignant tumor cells similar to fibrosarcoma and osteosarcoma. Case 2 was a 63-year-old male who died 11 years after the development of the initial symptom. At autopsy only sarcomatous tumors resembling malignant fibrous histiocytoma (MFH) were observed in the metastatic lesions. Both cases were treated with irradiation. It is suggested that the appearance of sarcomatous tumor in current two cases of chordoma might be due to the phenomenon of tumor progression closely associated with irradiation therapy. These two cases can be categorized as "chordoma with a malignant spindle cell component" in a sense that highly malignant sarcomatous components existed in conjunction with chordoma in the primary tumors.     

CHORDOMA – REPORT OF AN AUTOPSY CASE WITH FIBROSARCOMA

An autopsy case of sacrococcygeal chordoma associated with metastasizing fibrosarcoma developing adjacent to a recurrent chordoma was described.
The fibrosarcoma developed within one year after radiotherapy with 5,400 rads for the chordoma, but this is too short of a period for development of fibrosarcoma as a result of the irradiation.
In several literatures chordomas accompanying sarcoma or sarcomatous growth as malignant change have been reported. In this case both tumors were separated distinctly from each other by a connective tissue. Since no findings suggesting that chordoma had developed into fibrosarcoma could be obtained, we considered these as primary unrelated neoplasms.     

Malignant mesothelioma of the peritoneum: case reports and immunohistochemical findings including Ki-67 expression

A malignant mesothelioma (MM) is an aggressive neoplasm, although some patients have shown long-term survival, and factors related to survival remain uncertain. We present three cases of MM of the peritoneum including autopsy results, in which factors related to long-term survival were investigated. Case 1 was a 69-year-old man who died 6 years after the initial diagnosis. In case 2, a 67-year-old woman came to us with abdominal distention and, despite chemotherapy, died 9 months after the initial diagnosis. The patient in case 3 was a 68-year-old man who also had abdominal distention and died 9 months after the initial diagnosis. We studied the clinicopathological appearance and performed immunohistochemical staining including Ki-67 labeling index (Ki-67 LI) in primary and metastatic sites of these cases. The histological findings of case 1 indicated epithelioid type; case 2 and 3 were of biphasic type. Immunohistochemical results were consistent with MM. The Ki-67 LI value for both primary and metastatic sites of case 1 was significantly lower than those in cases 2 and 3. We consider Ki-67 LI to be a useful prognostic indicator for MM of the peritoneum.     

True carcinosarcoma of the maxillary sinus

A case of true carcinosarcoma primarily arising in the right maxillary sinus is reported in a 60-year-old male. His chief complaints were right nasal obstruction and bleeding. Histological findings of the biopsied primary tumor revealed two components of keratinizing squamous cell carcinoma and osteosarcoma which were intricately intermingled. Despite intensive irradiation and chemotherapy, and total maxillectomy, he finally died of rapid tumor recurrence and widely spreading metastases to lungs, pleurae and brain two months later. At autopsy the recurrent and metastatic tumors consisted entirely of the osteosarcoma component, suggesting the efficiency of the radiotherapy and chemotherapy against the carcinomatous component, but not against the sarcomatous one. As for histogenesis, this case was compatible with a combination tumor judging from histologic, immunohistochemical and electron microscopic findings.     

Incipient chordoma: a report of two cases of early-stage chordoma arising from benign notochordal cell tumors.

Chordomas are rare malignant bone tumors primarily involving both ends of the axial skeleton that present as destructive bone lesions with a large soft tissue mass. Chordomas were previously believed to arise from notochordal remnants. However, recent studies suggest the possibility that chordomas arise from benign notochordal cell tumors. We present two cases of coccygeal incipient chordoma that strengthen the new hypothesis. The first case was an 83-year-old man who died of prostatic adenocarcinoma. The second case was a 79-year-old man who died of hepatocellular carcinoma. The coccygeal tumors were composed of intraosseous and extraosseous infiltrative lesions. The intraosseous lesions consisted of both benign notochordal cell tumor and incipient chordoma. The extraosseous lesions were consistent with incipient chordoma. In addition, two other small benign notochordal cell tumors were found at a different level in case 1. It is conceivable that pre-existing intraosseous benign notochordal cell tumors transform into incipient chordoma and then extend through the cortex into the surrounding soft tissue. The incidence of incipient chordoma appears much higher than expected because chordomas are rare tumors with an incidence of one case per 1 000 000 persons per year. We suspect that unknown factors transform incipient chordoma into classic chordoma.     

Von Recklinghausen's disease (neurofibromatosis) associated with malignant pheochromocytoma

An autopsy case of von Recklinghausen's disease (vRD) associated with malignant pheochromocytoma is reported. The patient is a 36-year-old Japanese male and diagnosed as vRD both clinically and pathologically. He died from right adrenal tumor with wide spread metastases to lungs and bone marrow. The tumors presented satisfactory histological features in favor of pheochromocytoma and neurosecretory granules were demonstrated in both primary and metastatic lesions ultrastructurally. Statistical study of 182, 673 autopsy cases from Annuals of Japanese Autopsy Cases was also done in order to investigate the relationship between vRD and associating tumors including benign and malignant pheochromocytoma. Cases with vRD showed significantly higher incidences of malignant Schwannoma, neurofibrosarcoma, intracranial glioma, and pheochromocytoma compared to that of non-vRD cases. Other malignancies revealed rather smaller incidences than non-vRD cases. These neurogenic tumors are to be principal life threatening problems in patients with vRD. Rare incidence of malignant pheochromocytoma in vRD is to become from low incidence of pheochromocytoma, though significantly greater than that of non-vRD cases.     

Primary pulmonary artery sarcoma. Report of two autopsy cases studied by immunohistochemistry and electron microscopy, and review of 110 cases reported in the literature

Two cases of primary pulmonary artery sarcoma are reported. The patient in the first case was a 61-year-old male with a two-year history of cough and exertional dyspnea, who died of intractable cardiac failure two months after admission without establishment of a diagnosis related to the etiology of cardiac failure. Autopsy revealed a sessile tumor within the pulmonary trunk and a solitary metastatic lesion in the lung. Histologic, immunohistochemical and electron microscopic studies were performed and a diagnosis of malignant mesenchymoma was made. The patient in the second case was a 32-year-old male complaining of exertional dyspnea and back pain. Radiologic studies indicated a mediastinal tumor involving the pulmonary artery. Exploratory thoracotomy revealed that the mediastinal mass arose from the left pulmonary artery. He died of respiratory failure 26 months after onset of his initial symptoms. Histologic, immunocytochemical and electron microscopic studies of both surgical and autopsy materials revealed a malignant fibrous histiocytoma. One hundred ten previously reported cases of this tumor are reviewed, and its clinicopathologic and morphologic features and probable histogenesis are discussed.     

Mixed epithelial and stromal tumor of kidney with malignant transformation: report of two cases and review of literature

We present 2 cases of mixed epithelial and stromal tumor of the kidney with sarcomatous transformation. One patient was a 53-year-old woman who presented with macroscopic hematuria. The resected tumor involved the right renal parenchyma, measuring 13.0 x 8.0 x 4.0 cm, and extended to perirenal adipose tissue. The second patient was a 56-year-old woman who presented with right flank colic pain. The tumor measured 6.0 x 5.5 x 4.0 cm, with an intact capsule at the upper pole. Both tumors showed a well-circumscribed, multilocular, cystic, and focally solid mass. Sections of both tumors revealed benign and malignant components. The benign component consisted of multilocular cysts and fibrous stroma with a focally ovarian stromalike component. The malignant component in both cases was predominantly composed of undifferentiated cellular spindle cell sarcoma with frequent mitoses. One case showed additional heterologous malignant elements, including rhabdomyosarcomatous, chondrosarcomatous, and focal carcinomatous components. We report 2 additional cases of sarcomatous transformation in mixed epithelial and stromal tumor of the kidney.     

Metastatic Leydig cell tumor with sarcomatoid differentiation.

Leydig cell tumors of the testis are uncommon. Only about 10% of cases have a malignant course. It has been stated that the only definite criterion for malignancy is presence of metastasis. We present a 47-year-old patient with metastatic Leydig cell tumor 17 years after initial diagnosis, to our knowledge the longest reported interval between diagnosis and the development of metastasis. The primary tumor did not exhibit convincing features of malignancy. The initial metastasis in the right perirenal fat tissue showed a biphasic tumor with sarcomatoid differentiation not described previously in a metastatic Leydig cell tumor.     

Carcinosarcoma of the submandibular gland. An autopsy case.

We present a rare case of submandibular gland carcinosarcoma occurring in a 45-year-old male patient. His clinical history revealed that the carcinosarcoma had developed from a carcinoma ex mixed tumor in three years. In spite of repeated resection, intensive chemotherapy and irradiation, the tumor recurred and grew rapidly, and the patient died of hemothorax caused by rupture of a pulmonary metastatic tumor. The fourth recurrent tumor and autopsy specimens showed features of carcinosarcoma consisting of three tumor components, i.e., undifferentiated carcinoma, and chondrosarcomatous and osteosarcomatous growth. The metastatic nodules in both lungs and pulmonary hilar lymph nodes showed the same pattern. Immunohistochemically, the chondrosarcomatous cells were positive for vimentin and S-100 protein, and for epithelial markers such as epithelial membrane antigen (EMA) and cytokeratin (MA-902). Undifferentiated carcinoma cells, on the other hand, were partially positive for muscle actin other than cytokeratin (KL 1). Ultrastructurally, desmosome-like structures were seen in the chondrosarcomatous cells. These findings suggest that the sarcomatous lesions might have originated from epithelial cells.     

Sarcomatoid carcinoma of the urinary tract

Sarcomatoid carcinoma is a rare variant of malignant tumor arising from the urinary tract. This tumor had been termed carcinosarcoma because of its carcinomatous and sarcomatous components. There is still some confusion in the terminology between true carcinosarcoma and sarcomatoid carcinoma; however, the latter is now regarded as primarily a malignant epithelial tumor with pseudosarcomatous transformation.
Four cases of sarcomatoid carcinoma arising from the urinary tract are reported. The patients were a 77 year old female, and three males aged 62, 69 and 80 years. All but the eldest patient complained of gross hematurla. Surgical removal was performed in the younger three cases, and an autopsy was done in the remaining case. All the tumors were macroscopically polypoid. Histopathologic examination revealed fasciculated spindle-cell tumors with myxold stroma or malignant fibrous histiocytoma-like spindle cell tumors. The epithelial nature was proven in these sarcomatous cells by immunohistochemical andlor electron-microscopic examinations. Only a small amount of squamous cell carcinoma components was also evident in the latter three cases. Although the younger three patients were alive at 44, 23 and 39 months'follow-up, respectively, constant careful monitoring Is recommended.     

Primary carcinosarcoma of the vagina

Primary carcinosarcoma of the vagina is a very rare tumor, with only eight cases diagnosed as carcinosarcoma in the literature that we are aware of. We recently encountered a case of primary carcinosarcoma of the vagina in a 75-year-old woman. The patient had a history of hysterectomy and bilateral ovariectomy for uterine corpus cancer at 55 years of age. Recurrence of the cancer was suspected 17 years after the operation and irradiation therapy was performed, but the patient died 3 years after the recurrence. Autopsy revealed a mass lesion in the pelvic cavity that originated in the vagina. Histological examination showed that the tumor contained anaplastic carcinoma, rhabdomyosarcoma, leiomyosarcoma and chondrosarcoma components, and it was diagnosed as carcinosarcoma. The histological diagnosis of the uterine corpus cancer was well-differentiated adenocarcinoma, and there was no sarcomatous component. The carcinosarcoma occurred 17 years after the hysterectomy, and it was concluded to be a primary carcinosarcoma of the vagina. This is the first case of primary vaginal carcinosarcoma in which the epithelial and sarcomatous components were clearly identified histologically and immunohistochemically.     

PRIMARY PULMONARY ARTERY SARCOMA. Report of Two Autopsy Cases Studied by Immunohistochemistry and Electron Microscopy, and Review of 110 Cases Reported in the Literature

Two cases of primary pulmonary artery sarcoma are reported. The patient in the first case was a 61-year-old male with a two-year history of cough and exertional dyspnea, who died of intractable cardiac failure two months after admission without establishment of a diagnosis related to the etiology of cardiac failure. Autopsy revealed a sessile tumor within the pulmonary trunk and a solitary metastatic lesion in the lung. Histologic, immunohistocyto-chemical and electron microscopic studies were performed and a diagnosis of malignant mesenchymoma was made. The patient in the second case was a 32-year-old male complaining of exertional dyspnea and back pain. Radiologic studies indicated a mediastinal tumor involving the pulmonary artery. Exploratory thoracotomy revealed that the mediastinal mass arose from the left pulmonary artery. He died of respiratory failure 26 months after onset of his initial symptoms. Histologic, immunocytochemical and electron microscopic studies of both surgical and autopsy materials revealed a malignant fibrous histiocytoma. One hundred ten previously reported cases of this tumor are reviewed, and its clinicopathologic and morphologic features and probable histogenesis are discussed. ACTA PATHOL JPN 38: 883∼896, 1988.     

SARCOMATOUS VARIANT OF MALIGNANT HISTIOCYTOSIS; A CASE REPORT and REVIEW OF THE LITERATURE

The case is that of a 21-year-old female with mediastinal and subcutaneous, tumors composed of sarcomatous growth of poorly differentiated histiocytes defined by enzyme histochemical staining, ultrastructural observation and detection of surface markers. At autopsy neoplastic cells in the solid tumor became less cohesive and pleomorphic with erythrophagia, while features in the lymph node draining from the tumor was compatible with the criteria of malignant histiocytosis. The place where such a sarcomatous variant of malignant histiocytosis should be placed within other histiocytic tumors is discussed and the literature reviewed.     

Endometrial carcinoma recurring as carcinosarcoma: report of two cases

Endometrial carcinosarcoma is a rare, aggressive disease, accounting for approximately 3% of all uterine neoplasms. The emergence of sarcomatous elements is considered the evolution of subclones arising from high grade endometrial carcinomas. Here, we report two cases of primary endometrial carcinomas recurring as carcinosarcoma. Case 1. a 58-year-old postmenopausal woman diagnosed to have a poorly differentiated endometrial endometrioid adenocarcinoma (FIGO stage IB) developed an intra-abdominal recurrence of disease after 17 months from diagnosis. Histopathological analysis documented a biphasic neoplasia consisting of an epithelial (grade 3 endometrial endometrioid adenocarcinoma) and a sarcomatous component. Salvage chemotherapy with cisplatin, ifosfamide, epirubicin, and then with taxotere was attempted. The patient died after 2 months. Case 2. A 56-year-old woman with a diagnosis of grade 3 endometrial adenosquamous carcinoma of the endometrium (FIGO stage IIIA) experienced pelvic recurrence after five months from completion of chemotherapy. Definitive histology was malignant mixed mesodermal tumor with focal areas of chondrosarcomatous elements. The patient was triaged to exclusive concomitant chemoradiotherapy and salvage chemotherapy. The patient died after 3 months. We describe two cases of high grade endometrial carcinomas recurring as carcinosarcoma, thus providing evidence that the metaplastic sarcomatous evolution is a very rare event which can occur in patients with anaplastic endometrial cancer.     

Primary bone carcinosarcoma: chondrosarcoma and squamous cell carcinoma with keratin pearl formation

Malignant bone tumors with epithelial differentiation are extremely rare. Only one case of primary malignant bone tumor with distinct squamous cell carcinoma and chondrosarcoma has ever been reported. Reported herein is a case of primary malignant bone tumor with distinct squamous cell carcinoma and chondrosarcoma, so-called carcinosarcoma of bone, arising in the femur of a 53-year-old man. The tumor was located within the femur and was diagnosed by curettage as a well-differentiated chondrosarcoma. No primary tumor was detected in any other organ. Within a few months the tumor had rapidly grown toward the soft tissue, and hemipelvectomy was performed. Examination of the surgical specimen revealed that the tumor was mainly composed of undifferentiated spindle sarcoma cells with scattered foci of chondrosarcoma and of squamous cell carcinoma with keratin pearl formation. The patient died approximately 6 months postoperatively. At autopsy multiple metastases were detected in the heart, both lungs, muscles, and lymph nodes. Interestingly, the chondrosarcoma and squamous cell carcinoma components were observed in several metastatic foci. The tumors in both the previously reported case and the present case contained components of chondrosarcoma and squamous cell carcinoma with keratin pearl formation, and this combination of histological features may be a unique characteristic of carcinosarcoma of bone.     

Renal oncocytoma

Up to date only five cases of renal oncocytomas have been reported in Japan. We describe two cases of renal oncocytomas. Case 1: A 63-year-old woman, who had no urological symptoms, was pointed out of a cystic mass in the left kidney by chance. A resected kidney had a cystic tumor, which was well encapsulated and measured 6 X 7 X 8 cm. The tumor was unilocular and thick-walled cyst filled with bloody fluid. Histologically, the tumor was composed of oncocytes with eosinophilic granular cytoplasms that were packed with mitochondria. Case 2: A 70-year-old women died of malignant lymphoma and an autopsy was performed. The tumor was found in the right kidney by chance and measured 1 cm in diameter. The cut-surface of the tumor was uniform and tan-brown in color. Histologically, the tumor was composed of cells same as Case 1. We discuss the incidence of this tumor and the morphological characteristics of our cases.     

Bone metastasis from malignant phyllodes breast tumor: report of two cases

Background

Phyllodes tumors are rare fibroepithelial tumors accounting for less than 1 % of all breast neoplasms. They are malignant in 20 % of cases. Only a few cases of malignant phyllodes tumors metastatic to bone have been reported.

Case presentation

Case 1: A 40 year-old white woman presented with three-week history of pain and functional impairment of the left lower limb. Her clinical past was remarkable for previous left mastectomy and radiotherapy for malignant phyllodes tumor performed one year ago. Computed tomography revealed a moth-eaten appearance of the left femoral head. The patient underwent computed guided femoral head biopsy. Pathological findings were consistent with metastatic malignant phyllodes tumor. The patient received ifosfamide and adriamycin chemotherapy. She is doing well without any evidence of progression on her imaging follow- up after 8 months.Case 2: A 48 year-old white woman, with history of bilateral mastectomy and radiotherapy for malignant phyllodes tumor performed one and two year ago, presented with four-week left lower quadrant abdominal pain. Computed tomography and magnetic resonance imaging revealed a solid aggressive osteolytic mass of the left iliac bone with extensive soft tissue invasion. Biopsy of the tumor was performed and showed a sarcomatous proliferation consistent with metastatic malignant phyllodes tumor. The patient received the same chemotherapy regimen as in the first case but without any response on her imaging follow up after 6 months.

Conclusion

Malignant phyllodes tumor is a rare and aggressive fibroepithelial neoplasm. An accurate diagnosis of metastases should be based on clinicopathological correlation allowing exclusion of differential diagnoses. The goal of successful managing this tumor is early detection and complete resection prior to dissemination.

     

CILIATED-CELL ADENOCARCINOMA OF THE PANCREAS

An autopsy case of ciliated-cell adenocarcinoma of the pancreas was reported. A 65-year-old man, who had undergone subutotal gastrectomy because of advanced gastric carcinoma 6 years previously, died of obstructive jaundice. The autopsy revealed a primary tumor in the head of the pancreas and multiple metastatic foci in the liver, lungs, and regional lymph nodes. Histologically, the pancreatic tumor was moderately differentiated papillary adenocarcinoma with well developed cilia. The metastatic tumor also showed similar histology. Histologic and ultrastructural features of the tumor were described in detail and the cytogenesis of ciliated-cell adenocarcinoma was briefly discussed.     

Ciliated-cell adenocarcinoma of the pancreas

An autopsy case of ciliated-cell adenocarcinoma of the pancreas was reported. A 65-year-old man, who had undergone subtotal gastrectomy because of advanced gastric carcinoma 6 years previously, died of obstructive jaundice. The autopsy revealed a primary tumor in the head of the pancreas and multiple metastatic foci in the liver, lungs, and regional lymph nodes. Histologically, the pancreatic tumor was moderately differentiated papillary adenocarcinoma with well developed cilia. The metastatic tumor also showed similar histology. Histologic and ultrastructural features of the tumor were described in detail and the cytogenesis of ciliated-cell adenocarcinoma was briefly discussed.