Liposarcoma of pleural cavity with recurrence as malignant fibrous histiocytoma

The case of a 54-year-old male, with a massive right pleural liposarcoma weighing over 3200 g, is presented. The tumor was found by light and electron microscopy to be of well-differentiated and pleomorphic subtypes, and it apparently represents the sixth reported case of liposarcoma primary to the pleura. Two years after excision of the primary tumor, it recurred as a neoplasm with histologic and ultrastructural features characteristic of malignant fibrous histiocytoma. The histogenetic and pathologic implications of the above findings are discussed, and the literature regarding intrathoracic liposarcoma and malignant fibrous histiocytoma is reviewed.     

Use of antibodies to intermediate filaments in the diagnosis of metastatic amelanotic malignant melanoma

Immunofluorescent staining of tissue from a lung tumor detected 12 years after excision of a primary malignant melanoma of the skin was negative for prekeratin and positive for vimentin, indicating that the tumor was not epithelial in origin and excluding carcinoma from the differential diagnosis. Complementary conventional staining with hematoxylin-eosin confirmed the melanocytic origin of the tumor, indicating that it was probably an amelanotic metastasis of the original malignant melanoma. The findings in this case demonstrate the potential usefulness of immunohistochemical microscopic characterization of specific intermediate filament proteins in the diagnosis of otherwise ambiguous cases of amelanotic melanoma.     

Inguinal lymph nodal metastasis of myxopapillary ependymoma confirmed by fine-needle aspiration cytology, biopsy, and immunohistochemistry: case report

Ependymoma (EP) rarely metastasizes outside the central nervous system. Inguinal nodule metastasis of EP more than 10 years after surgical resection and radiotherapy is extremely rare. We report a man aged 38 years who underwent surgery for lumbosacral myxopapillary EP at the age of 22 years and was treated with several cycles of radiotherapy. The patient was reoperated for residual tumor and received two complete cycles of radiotherapy for 11 years. Biopsies were always diagnosed as myxopapillary EP. Five years after the last surgical excision, the patient developed abdominal pain and inguinal lymphadenopathy. Biopsy was performed by fine-needle aspiration and was proven malignant epithelial neoplasm with a myxoid background, was diagnosed as metastasis of EP. Biopsy showed an anaplastic EP grade III. EP is often recurrent at the primary site but can seed on the entire cerebrospinal axis. We describe the clinical features of this rare lesion and particularly emphasize the need for long-term follow-up, for more than 10 years after the initial treatment, in patients with EP and malignant transformation after radiotherapy.     

Primary cutaneous leiomyosarcoma: a histological and immunohistochemical study of 4 cases

Primary cutaneous leiomyosarcoma is an uncommon malignant neoplasm with a predilection for the lower extremities. A retrospective study of 4 cases was undertaken to analyse the clinicopathological characteristics and immunohistochemical profile of these neoplasms with emphasis on prognosis. Two male and 2 female patients aged between 49 and 80 years presented with painless tumours involving the lower lip, the chin, the scrotum and the shoulder. Histological examination of the initial biopsy specimen established a diagnosis of cutaneous leiomyosarcoma. All cases co-expressed smooth muscle actin and vimentin regardless of primary tumour site. Wide surgical excision of the tumour was performed in only 3 cases, and the remaining patient refused further treatment. Of the patients undergoing surgical intervention, local recurrence occurred in one case. No metastases were observed. Long-term follow-up of patients with cutaneous leiomyosarcoma is mandatory to detect local recurrence and distant metastases that can occur even years after the initial excision.     

Malignant fibroxanthoma (malignant histiocytoma): an ill-defined histologic entity with unpredictable biologic behavior

Summary The case of a 45-year-old woman with malignant fibroxanthoma (malignant histiocytoma) of the abdominal wall is reported. A local recurrence five years after surgery and radiation therapy required removal. Subsequent surgical intervention for radionecrosis and a fecal fistula failed to reveal residual tumor; the patient is free of tumor twenty-five years after the initial excision. The differential diagnosis of atypical and malignant fibroxanthoma is discussed. Studies of the small number of malignant fibroxanthomas reported in the literature suggest a poor correlation of the histopathologic characteristics of these lesions with their relatively benign biologic behavior.     

Primary kaposiform hemangioendothelioma of a long bone: two cases in unusual locations with long-term follow up

Kaposiform hemangioendothelioma (KHE) is a rare vascular neoplasm of low malignant potential that mainly affects infants and adolescents. The tumor almost exclusively occurs in somatic soft tissue or the retroperitoneum. We report herein two cases of primary KHE occurring in a long bone without cutaneous changes with long-term follow up in young patients. The patients were a 9-year-old girl and 5-year-old boy presenting with lytic lesions of the femur and humerus, respectively, without cutaneous lesions. Histologically, the neoplasms were comprised of nodules of spindle- to oval-shaped cells growing in an infiltrative fashion. The neoplastic cells formed poorly canalized or slit-like blood vessels alternating with solid spindle areas. Immunohistochemical studies showed that the tumor cells expressed CD31, CD34 and Fli1, but not HHV8, LNA-1 or GLUT1. D2-40 stained the neoplastic spindle cells and lymphatic channels adjacent to vascular lobules. The girl remains well with 15 years and 6 months follow up after a second complete excision. The boy has no signs of recurrence or metastasis nearly 5 years after local complete excision. To our best knowledge, this is the first report in the English literature of primary long bone occurrences of KHE without cutaneous changes with long-term follow up.     

Primary peritoneal, omental, and mesenteric tumors in childhood

Of 21 primary peritoneal, omental, and mesenteric tumors, 14 were cysts and seven were solid. Most of the cysts were omental-mesenteric lymphangiomas; the sole exception was a cystic mesothelioma in a newborn. The solid tumors included three multicentric hamartomas, three sarcomas of undetermined histogenesis, and one immature teratoma. Since cystic lymphangiomas carry a good prognosis following surgical excision, they must be distinguished from cysts of mesothelial origin. Solid hamartomas may simulate malignant tumors because of multicentricity and immature tissue composition. Although peritoneal sarcomas of undetermined histogenesis are apt to be large, composed of primitive cells, and disposed to the development of implants, current treatment modalities may control the disease. One of three treated patients survived for 8 years. A child with a solid immature teratoma without a malignant germ-cell component remains free of disease 5 years after diagnosis. Our review, which represents the combined experience of two children's hospitals over several decades, did not include tumors such as malignant mesothelioma, lipoma, liposarcoma, leiomyoma, mesenchymoma, fibromatosis, and other neoplasms, which may rarely arise in the peritoneum, omentum, and mesentery of children.     

Silver-stained nucleoli and nucleolar organizer region counts are of no prognostic value in thick cutaneous malignant melanoma

Nucleolar organizer regions (NORs) can be stained by a simple one-step silver technique; the black dots formed are termed AgNORs. Often AgNORs are tightly clustered, appearing as one silver-stained nucleolus (AgNu). We have assessed this technique as a possible prognostic indicator for thick (greater than 3.0 mm) primary cutaneous malignant melanoma (CMM). Three groups were studied: (A) seven thick CMM that had not metastasized 8-20 years after excision; (B) three thick CMM that developed metastases 6-9 years after excision; and (C) twelve CMM that presented with metastases or developed them within 4 years of excision. Two methods of counting silver-stained black dots in nuclei were employed: one method counted easily discernible black dots consisting of AgNus and dispersed AgNORs; the other attempted to count actual AgNORs both dispersed and clustered within AgNus. Scores per nucleus by the first method were 1.5-6.7 in group A, 1.1-2.6 in group B, and 1.4-5.4 in group C. AgNOR counts by the second method were 6.2-13.0 in group A, 5.4-8.9 in group B, and 5.3-10.5 in group C. No significant difference was present between groups for scores by either method. Due to the subjectively, technical difficulty, non-reproducibility, and tedium associated with the second method of attempting to count individuals AgNORs, the first method is recommended. It is concluded that this technique is of no value in predicting prognosis for CMM.     

Primary sclerosing epithelioid fibrosarcoma of the sacrum: a case report and review of the literature

Sclerosing epithelioid fibrosarcoma is a rare tumour characterised histologically by a predominant population of epithelioid cells arranged in strands and nests, embedded in a fibrotic and hyalinised stroma. It is a low grade tumour with an indolent course. A 48 year old woman presented with a painful swelling over her back for six months. Investigation and biopsy revealed features of sclerosing epithelioid fibrosarcoma involving the left half of the sacrum, left sacro-iliac joint, and posterior part of the left ilium. Preoperative radiotherapy and wide location excision of the tumour were followed by metastatic recurrence of the tumour in the lung and scalp six years after initial presentation. The tumour showed typical histology of sclerosing epithelioid fibrosarcoma. The radiological features confirmed its primary location in the sacrum. The patient declined chemotherapy and died of disseminated disease eight years after initial presentation. Review of the literature confirms the fact that sclerosing epithelioid fibrosarcoma, despite its low grade, is a clinicopathologically distinct tumour with full malignant potential, the recurrence, metastasis, and mortality rate being 48%, 60%, and 35%, respectively. Sclerosing epithelioid fibrosarcoma can occur as a primary bone tumour, the clinical behaviour of which is probably similar to its soft tissue counterpart.     

Multimodality management of a case of primary osteogenic sarcoma of the zygoma

Craniofacial osteogenic sarcomas are rare primary malignant bone tumors and very few cases involving zygomatic bone were reported in literature. We present our experience of multimodality management of a case of primary osteogenic sarcoma of zygoma. Wide radical excision of the tumor including the parotid gland was done followed by three cycles of adjuvant chemotherapy and fifty Gy of external radiotherapy. The patient is disease-free at two years follow-up. Till 1970s, craniofacial osteogenic sarcomas were managed mainly by radical surgery with a high local failure rate. With the advances made in the field of radiotherapy and chemotherapy, multimodality therapy is playing a major role in the treatment of these aggressive tumors with better overall and disease-free survival.     

Trichoblastic carcinoma ("malignant trichoblastoma") with lymphatic and hematogenous metastases.

We report an aggressively behaving malignant trichogenic tumor arising in a trichoblastoma (TB) with widespread lymphatic and hematogenous metastases in a 55-year-old man with a concomitant B-cell chronic lymphocytic leukemia. The primary tumor had been present and unchanged for as long as 40 years before excision. Typical trichogenic TB with dystrophic calcification and even ossification was still present peripheral to the malignant transformation. The malignant neoplasm consisted of basaloid cells, spindle cells arranged in fascicles and densely packed rounded nests or "cell balls." The metastases consisted of immature basaloid cells and cell balls, and the recurrences became successively more undifferentiated. The residual TB reacted with antibodies to cytokeratin (CK) 6, 8, 14, and 17 and focally to S-100; the malignant primary tumor reacted uniformly with antibodies to vimentin and only focally with antibodies to CK and S-100. The metastatic tumor had lost epidermal CK expression but maintained expression of S-100 in paraffin-embedded tissues. Trichoblastic differentiation was confirmed in frozen tissues with antibodies to hair keratins. No expression of p53 or bcl-2 was identified, but p-glycoprotein (MDR-1 gene related) was expressed by primary and metastatic tumor cells. We believe that this neoplasm is best classified as a trichoblastic carcinoma arising in a TB in association with a B-cell chronic lymphocytic leukemia. This case illustrates that TBs have the potential for malignant transformation and aggressive behavior.     

Extramammary Paget's disease with aggressive behavior: a report of two cases.

Extramammary Paget's disease (EMPD) is an intraepithelial neoplastic disorder which is included as a rare malignant condition. However, it sometimes shows aggressive behavior of local recurrence and coexisting malignancy. We had experienced nine cases of EMPD involving the scrotum for seven years. Two cases of them presented metastasis. The first case presented extensive inguinal lymph node metastasis with underlying adnexal adenocarcinoma one year after wide local excision. The second case initially presented multiple metastasis to the liver and in the lymph node. The latter, showing fulminant progression with liver metastasis, may be only the second case reported in English literature. EMPD is considered as a malignant neoplasm with aggressive behavior from initial presentation. Because wide local excision of the lesion alone may be occasionally insufficient, a careful follow-up must be done to detect recurrence or internal malignancy.     

Primary cardiac tumors: 32 years of experience from a Spanish tertiary surgical center

IntroductionPrimary cardiac tumors are rare entities with an autopsy frequency ranging between 0.001% and 0.3%. Our objective was to review 32 years of experience from a Spanish tertiary surgical center.MethodWe collected data on 73 patients with a histopathological diagnosis of a primary cardiac tumor in a retrospective analysis from 1979 to 2012. Tissue samples were obtained either at surgery or from necropsy and subsequently divided into benign and malignant groups.ResultsBenign neoplasms accounted for 84.9% of cases. The average age at diagnosis was 61 years, and tumors were twice as frequent among women. It was an incidental finding in one quarter of patients. In symptomatic patients, the typical presentation was of cardioembolic stroke or of congestive symptoms. Myxoma (93.5%) was the most common diagnosis, typically affecting the left atrium (74.2%). Surgical resection was curative for 95% of these patients. Malignant tumors represented 15.1% of cases with an average age at diagnosis of 50 years. Over 90% of these patients were symptomatic at presentation with the cardinal symptom being heart failure. Undifferentiated sarcoma was the most frequent malignant tumor (36.4%). Less than 20% of patients were alive 1 year after diagnosis despite the treatment.ConclusionsIn our study, primary cardiac tumors were insidious. The most common was left atrial myxoma in which surgical resection was considered curative. Undifferentiated sarcoma was the most frequent malignant tumor. Malignant neoplasms carried a worse prognosis with unsuccessful total excision and the presence of metastatic disease being markers of an adverse outcome.     

Tumor implantation along abdominal trocar site after pelviscopic removal of malignant ovarian tumor--a case report.

The application of pelviscopic surgery for the management of ovarian tumors has increased dramatically in the last few years. Of particular concern is the pelviscopic excision of malignant ovarian tumors. One of the important potential problems with this approach is disseminating malignant cells to peritoneal surface. The aim of this report is to draw attention to the possibility of the occurrence of a tumor implantation at the pelviscopic port site in patients with malignant ovarian tumors. A case is presented here in which a localized tumor implant occurred in the abdominal trocar site after pelviscopic removal of ovarian mass subsequently found to be squamous cell carcinoma arising in mature cystic teratoma with brief review of literatures.     

Mortality and morbidity after initial diagnostic excision biopsy of cutaneous melanoma in primary versus secondary care

Background

Current UK melanoma guidelines do not support the initial diagnostic excision biopsy of pigmented lesions in primary care, although this is standard in other countries such as Australia. Previous research in Northeast Scotland found that initial diagnostic excision biopsies in primary care that prove to be melanoma were no more likely to be incomplete than those performed in secondary care, but data on longer-term outcomes were not available.

Aim

To determine whether initial diagnostic excision biopsy of cutaneous melanoma in primary versus secondary care leads to poorer survival and increased morbidity.

Design and setting

Analysis of a linked dataset comprising pathological data from melanoma cases diagnosed in Northeast Scotland between 1991 and 2007, the General Registry Office (Scotland) death registry, and an NHS Scotland episode of care database.

Method

Patient data from three sources were matched using the Community Health Index (CHI) number. Cox proportional hazards regression, with robust standard error estimates, was used to examine the hazard ratio (95% confidence interval) of key mortality and morbidity outcomes based on excision in primary versus secondary care. Analysis was conducted before and after adjustment for operator and patient-level factors, using a multilevel approach.

Results

Patients receiving their initial diagnostic excision biopsy for melanoma in primary versus secondary care were no more likely to be dead, or to have died of metastatic malignant melanoma. Patients who had their initial diagnostic excision biopsy for melanoma in primary care had significantly fewer subsequent hospital admissions and spent fewer days in hospital.

Conclusion

These findings suggest that initial diagnostic excision biopsy of melanoma in primary care does not lead to poorer long-term outcomes.     

Mesenchymal renal tumors in infancy: a reappraisal

Eight cases of primary mesenchymal renal tumor in infants under one year of age were studied. The patients' ages ranged from one day to seven months; all but one were diagnosed within the first three months of life. There were four typical congenital mesoblastic nephromas. Two malignant mesenchymal, one intermediate case of difficult classification designated as "cellular variant" of congenital mesoblastic nephroma, and one hemangioendothelioma. All patients were alive and free of tumor 3 9/12 to 18 10/12 years after surgery. Of the four infants with congenital mesoblastic nephroma, one was treated by tumor excision alone; nearly 13 years later this patient was free of tumor. A high degree of cellularity and a high nucleus-cytoplasm ratio were features that characterized the tumor diagnosed as "cellular variant" of congenital mesoblastic nephroma. A sarcomatous gross appearance with cavitation necrosis was seen in the two instances of malignant mesenchymal nephroma; one of these metastasized to the lung, whereas in the other, though no metastases developed, all the histologic details of the previous case were reproduced, including distinct foci of necrosis and a high mitosis rate. Mesenchymal renal tumors in young infants constitute a set more heterogeneous than has been previously assumed. They should not be considered uniformly benign. As a group, they span the whole spectrum between benign, morphologically quiescent lesions, clinically and pathologically intermediate or indeterminate ones, and outright malignant tumors with a high risk of distant spread.     

Genetics of metastasis: melanoma and other cancers

Melanoma is a malignant neoplasm of melanocytes that accounts for the majority of skin cancer deaths despite comprising less than 5% of all cutaneous malignancies. Its incidence has increased faster than that of any other cancer over the past half-century and the annual costs of treatment in the United States alone have risen rapidly. Although the majority of primary melanomas are cured with local excision, metastatic melanoma historically carries a grim prognosis, with a median survival of 9 months and a long-term survival rate of 10%. Given the urgent need to develop treatment strategies for metastatic melanoma and the explosion of genetic technologies over the past 20 years, there has been extensive research into the genetic alterations that cause melanocytes to become malignant. More recently, efforts have focused on the genetic changes that drive melanoma metastasis. This review aims to summarize the current knowledge of the genetics of primary cutaneous and ocular melanoma, the genetic changes associated with metastasis in melanoma and other cancer types, and non-genetic factors that may contribute to metastasis.     

Sacrococcygeal Chordoma with a Malignant Spindle Cell Component

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 as sociated 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.     

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.