Malignant Histological Features Research Articles

Lymphomatoide Papulose bei einem 2 1/2-jährigen Jungen

Lymphomatoid papulosis (LyP) is a rare cutaneous lymphoproliferative disorder that has malignant histologic features and a benign clinical course. LyP is classified according to the WHO/EORTC classification for cutaneous lymphoma as a CD30-positive lymphoproliferative disorder. Few previous reports have detailed features of LyP in pediatric and adolescent patients, but LyP very rarely presents in early childhood. A 2 1/2-year-old boy presented with a 1-year history of recurrent papular and nodular lesions on the face and trunk. Clinical and histopathologic criteria lead to the diagnosis lymphomatoid papulosis.

Genetic findings in small melanocytic lesions

AbstractPurpose The management of patients with small melanocytic choroidal tumour is controversial. The aim of this presentation is to inform the current debate on patient care by reporting on histological grade of malignancy and genetic type of such tumours.Methods We reviewed our database and selected patients with: a melanocytic tumour having dimensions conforming to the TNM size category 1 (i.e., ‘small’); tumour involving choroid; and clinical and/or histological features of malignancy. Patients with such a ‘small choroidal melanoma’ were excluded if not resident in the British Isles, if the tumour was not treated at our centre between 1993 and 2010 or if they had bilateral uveal melanoma.Results 3132 patients with a choroidal melanoma were identified, of which 1001 (32.2%) were ‘small’. Chromosome 3 status was known in 102 of such small choroidal melanomas. Chromosome 3 loss was detected in 40% of these. Approximately 63% of tumours with chromosome 3 loss showed chromosome 8q gain (cf. 85% of tumours of TNM size category 4). Approximately 70% (i.e., 16 tumours) of small choroidal melanomas with both these genetic abnormalities also showed epithelioid cells. Metastatic death occurred in 4 patients, all of whom had a tumour with chromosome 3 loss, chromosome 8q gain and epithelioid cells.Conclusion Our data suggest that uveal melanomas show ‘crescendo malignancy’ with cumulative genetic abnormalities ultimately resulting in a lethal ‘double‐hit’ of chromosome 3 loss and chromosome 8q gain. We hypothesize that treatment of small choroidal melanomas showing either chromosome 3 loss or chromosome 8q gain prevents such a lethal double hit and hence the onset of metastatic spread. Delayed treatment of small choroidal melanomas may therefore be dangerous. Prognostication is greatly enhanced by multivariate analysis combining TNM staging with histological grading and genetic typing also taking account of the patient’s age and sex. There would seem to be scope for greater use of biopsy so as to provide better counselling of patients and plans for systemic screening and other aspects of care.

Malignant Fat-Forming Solitary Fibrous Tumor (so-called “Lipomatous Hemangiopericytoma”)

Fat-forming solitary fibrous tumor is a rare variant of solitary fibrous tumor (SFT). Generally regarded as benign, very few fat-forming SFTs with malignant histologic features have been reported. Here, we report 14 histologically malignant fat-forming SFTs to better characterize this subset. Seven patients were female and 7 were male, with ages ranging 20 to 93 years (median, 57 y). Five tumors were located in the lower limb, 3 in the trunk, 3 in abdominopelvic locations, 2 in the head and neck region, and 1 in the upper limb. The tumor size ranged from 3.4 to 20 cm (median, 8.6 cm). Histologically, all exhibited at least focal hypercellularity; 12 tumors had mitoses >4/10 high-power fields (range, 2 to 37; median, 8), 12 showed at least moderate atypia, and 8 showed necrosis. It should be noted that 7 tumors contained only mature adipose tissue, whereas 5 contained multivacuolated lipoblasts and 2 had areas resembling atypical lipomatous tumor (ALT). Immunohistochemically, CD34 and CD99 were positive in most cases (11 of 14 and 8 of 10, respectively); MDM2 and CDK4 were both negative in all 4 cases tested (including both tumors with ALT-like areas). Follow-up data from 10 cases (median duration, 47.5 mo; range, 5 to 76) showed 2 patients with multiple metastases (both to lung and bones, and 1 each to breast and to soft tissue), both of whom died of disease. In conclusion, fat-forming SFTs exhibiting malignant histologic features have potential for aggressive behavior. The presence of lipoblasts and/or ALT-like areas, although described in some "benign" examples of fat-forming SFT, seems much more common in the malignant subset and may prompt a careful search for morphologic evidence of malignancy in any case of fat-forming SFT.

Rhabdoid papillary meningioma: A clinicopathologic case series study

World Health Organization (WHO) grade III meningiomas are subclassified on the basis of their architectural pattern into papillary and rhabdoid subtypes. Some meningiomas even combine papillary architecture with rhabdoid cytology. Additionally, they always show malignant histological features, follow an aggressive clinical course and tend to spread through the CSF after frequent local recurrence. We render the first series of rhabdoid papillary meningioma with review of the literature to further elucidate its biological behavior. From six patients (three male, three female), nine specimens of rhabdoid papillary meningioma were obtained between 1994 and 2010. Correlations of histologic parameters, immunohistochemical study, and clinical features were assessed. The mean age of patients was 44.7 years at their first operation. The mean postoperative follow-up period was 63.2 months. Five patients experienced tumor recurrence, and one of them died from the disease after diffuse leptomeningeal dissemination. The mean time to first recurrence was 28 months. Only one patient was free of tumoral recurrence after an 8-year follow-up. Immunohistochemically, all tumors were positive for vimentin and epithelial membrane antigen. MIB-1 labeling indices were higher following tumor recurrence. The present study expands the clinicopathologic horizon of rhabdoid papillary meningioma and suggests that it will behave aggressively based on its histology and concomitant features of atypia or malignancy or high MIB-1 labeling indices. Close follow-up and aggressive treatments of these tumors are warranted.

Clear cell ‘sugar’ tumour of the lung with malignant histological features and melanin pigmentation – the first reported case

Clear cell ‘sugar’ tumour of the lung with malignant histological features and melanin pigmentation – the first reported case

Mucoepidermoid Carcinoma of Tracheobronchial Tree: Clinicopathological Study of 31 Cases

BACKGROUNDAll aspects of mucoepidermoid carcinoma (MEC) of the lung including histologic grading, clinical behavior and its differentiation from adenosquamous cell carcinoma are still not fully understood.METHODSWe reviewed the hematoxylin-eosin stained slides and medical records of 31 cases of MEC of the lungs. The cases were classified as low and high grade according to the quantitative grading system formulated for MEC. High grade tumors were tested for an epidermal growth factor receptor (EGFR) mutation.RESULTSTwenty eight cases were classified as low grade and 3 cases as high grade. Histologically, lower glandular component, cellular atypia, necrosis, mitoses >4/10 high power fields, and endolymphatic tumor emboli were typical characteristics of a high grade tumor. Although some tumors showed histologic features mimicking high grade tumors, they were classified as low grade tumors according to this quantitative grading system. Low grade tumors showed no recurrence or metastasis. However, among three patients with a high grade tumor, two had distant metastases and one died of disease. Additionally, an EGFR mutation was not detected.CONCLUSIONSA high grade MEC was consistently different from a low grade tumor with regard to malignant histologic features and poor prognosis. Therefore, correct histologic grading is important in predicting the prognosis to avoid unnecessary treatment.

Clinicopathologic analysis of rhabdoid meningioma

Rhabdoid meningioma is an uncommon variant of meningioma, and was classified separately for the first time in the 2000 World Health Organization’s classification of tumors of the nervous system. Because it often shows malignant histological features and follows an aggressive clinical course, it has been classified as a grade III neoplasm. We describe the clinicopathologic features of 13 patients with this rare tumor. From 13 patients (seven male, six female), 19 specimens of rhabdoid meningioma were obtained between 2001 and 2009. The mean age of patients was 50.4 years at their first operation. The mean postoperative follow-up period was 35.7 months. Five patients experienced tumor recurrence, and two patients died from the disease. The mean time to first recurrence was 36.1 months. The recurrence-free survival rates at 1 and 5 years were 62% and 23%, respectively. Immunohistochemically, all tumors were positive for vimentin and epithelial membrane antigen. MIB-1 labeling indices were higher following tumor recurrence. Close follow-up and aggressive treatment of these tumors is warranted.

Sex, Age, and Surgeon Decision on Nephron-sparing Surgery Are Independent Predictors of Renal Masses With Benign Histologic Findings—A Multicenter Survey

To define the preoperative independent predictors indicating that renal mass has benign histologic features. A total of 1664 patients with Stage T1-T2N0M0 with a unilateral renal mass underwent nephrectomy. The endpoint at multivariate analysis was benign versus malignant histologic features. The surgical approach (odds ratio [OR] 2.9, P = .0001), sex (OR 1.97, P = .0001), and age (OR 1.01, P = .007) were independent predictors for the malignant-benign distinction. Malignant tumors were more likely to occur in men (878 of 1009, 87%) versus women (515 of 651, 79%; P <.001). A weak relationship was found between an increasing tumor size and malignancy risk in men only. High-grade renal cell carcinoma was more prevalent in men (31% versus 21%, P = .001). The histologic tumor types were distributed differently between the 2 sexes: 8% papillary renal cell carcinoma in women versus 16% in men, 86% and 78% clear cell renal cell carcinoma, 33% and 57% oncocytoma, and 40% versus 12% angiomyolipoma, respectively. The physician's preoperative judgment regarding tumor amenability for nephron-sparing surgery resulted in patient selection: 10% benign tumors for radical nephrectomy versus 25% for partial nephrectomy (P = .001) and 31% versus 20% high-grade tumors, respectively (P = .0001). Renal tumors were consistently benign in 20% of women, regardless of size. In contrast, in men, the malignancy risk increased slightly with tumor size. The surgeons' preoperative decision regarding nephron-sparing surgery caused a selection bias in favor of benign lesions, regardless of sex. Our findings support the possibility of basing treatment decisions on the preoperative biopsy findings. Such changes could alter current practice and limit treatment of histologically proven benign lesions to surveillance or ablation only.

Glioneuronal neoplasms with malignant histological features: a study of 36 cases

Glioneuronal neoplasms with malignant histological features: a study of 36 cases

Malignant solitary fibrous tumour of the orbit

We aimed to report a case of orbital solitary fibrous tumour (SFT) in a child and to review the relevant literature. We describe an SFT in a 13-year-old boy with a 1-month history of painless proptosis in the left eye. Magnetic resonance imaging revealed a well circumscribed mass filling most of the left intraconal orbit. The lesion was excised and histopathological examination revealed a malignant SFT. Postoperative follow-up for 18 months was uneventful. Malignant SFT of the orbit should be included in the differential diagnosis of paediatric orbital tumours. Complete surgical excision remains the preferred method of management and the longterm prognosis is guarded.

Posttreatment Recurrence of Malignant Brain Neoplasm: Accuracy of Relative Cerebral Blood Volume Fraction in Discriminating Low from High Malignant Histologic Volume Fraction

To determine the accuracy of relative cerebral blood volume (rCBV) fraction for distinguishing high-grade recurrent neoplasm from treatment-related necrosis (TRN) in enhancing masses identified on surveillance magnetic resonance (MR) images following treatment for primary or secondary brain neoplasm. This institutional review board approved and HIPAA-compliant retrospective study included 30 patients undergoing resection of recurrent enhancing mass appearing after treatment with surgery and radiation, with or without chemotherapy. The enhancing mass volume was manually segmented on three-dimensional T1-weighted images. The rCBV maps were created by using T2-weighted dynamic susceptibility contrast perfusion MR imaging and registered to T1-weighted images, and the fraction of enhancing mass with rCBV above a range of thresholds was calculated. A receiver operating characteristic (ROC) curve was created by calculating sensitivity-specificity pairs at each threshold for rCBV fraction (< or = 20% or > 20%) by using percentage of malignant features at histologic evaluation as the reference criterion. Relationships between rCBV and probability of recurrence were estimated by using logistic regression analysis. ROC analysis showed excellent discriminating accuracy of rCBV fraction (area under the ROC curve, 0.97 +/- 0.03 [standard error]) and high efficiency (93%) with an rCBV threshold of 1.8 times that of normal-appearing white matter. Logistic regression analysis showed that a unit increase of rCBV is associated with a 254-fold increase (95% confidence interval: 43, 1504, P < .001) of the odds that enhanced tissue is recurrence, adjusting for age, treatment, volume of enhancing tissue, and time to suspected recurrence. The fraction of malignant histologic features in enhancing masses recurring after treatment for brain neoplasms can be predicted by using the rCBV fraction, with improved differentiation between recurrent neoplasm and TRN.

Atypical pituitary adenoma with malignant features

Pituitary carcinoma is characterized by the presence of systemic or central nervous system metastases rather than malignant histological features, making it an anomaly amongst carcinomas. In contrast, aggressive or atypical pituitary adenomas often have a relatively bland histological appearance despite their malignant growth patterns. We now report a case of a 67-years-old male with a giant pituitary tumor with overt pathologic and phenotypic features of malignancy, but the absence of metastases, that evolved from a benign non-functioning gonadotroph macroadenoma treated 10 years earlier. This case represents the first report of a pituitary adenoma with overt malignant histology that does not meet criteria for classification as pituitary carcinoma, helping to complete the pathological spectrum of disease observed with pituitary tumors.

Pseudoangiomatous Stromal Hyperplasia (PASH) of the Breast With Foci of Morphologic Malignancy: A Case of PASH With Malignant Transformation?

Pseudoangiomatous stromal hyperplasia (PASH) is a benign proliferation of the hormonally responsive, specialized mammary stroma characterized by slit-like pseudovascular spaces lined by bland spindle cells. It is usually an incidental microscopic finding but in some cases it may present as a slowly growing mass. A malignant counterpart for this lesion has not been reported. We describe a case of PASH with foci of malignant histologic features presenting as a slowly growing mass in a 30-year-old woman. The previously reported variants of PASH and the other mammary stromal lesions related to PASH are also discussed. This is perhaps the first case of PASH with foci of malignant histologic features reported in the literature and represents a rare sarcoma derived from specialized hormonally responsive mammary stroma.

Dermal Squamomelanocytic Tumor: Neoplasm of Uncertain Biological Potential

We report a case of exceedingly rare cutaneous neoplasm with histological features of malignancy and uncertain biological potential. The nodular, darkly pigmented facial tumor with central exulceration, size 12 x 10 x 7 mm, of the skin 61-year-old man preauricular left was completely exised. Histologically tumor consists of atypical squamous cells, which express signs of moderate to significant pleomorphism, mitotically active, with foci forming of parakeratotic horn cysts ("pearls"). Characteristically tumor also consists of large number of atypical melanocytes with multifocal pattern, inserted between atypical squamous cells, and which contain large amount of dark brown pigment melanin. Immunohistochemically, squamous cells stain positively with keratin (CK116), melanocytes were stained with S -100 protein, HMB 45, and vimentin, but failed to stain with CK 116. To our knowledge this is the sixth reported case in world literature. The follow-up time of four years no evidence of recurrence or metastasis, similar all reported cases, but it is too short period in estimation to guarantee a benign course. However, it appears that this group of neoplasm may have different prognosis from pure squamous carcinoma or malignant melanoma.

Primary anaplastic ganglioglioma with a small-cell glioblastoma component

Gangliogliomas usually present as benign tumors corresponding to World Health Organization (WHO) Grade I. Very rarely, gangliogliomas show histological features of malignancy and are then classified as anaplastic gangliogliomas of WHO Grade III or IV. In most cases, anaplastic gangliogliomas developed after radiation therapy or progression from a pre-existing low-grade ganglioglioma. Here, we report the case of a 77-year-old male patient who was operated on a primary ganglioglioma with a highly anaplastic glial component corresponding to a small-cell glioblastoma. To our knowledge, this is the first reported case of a primary anaplastic ganglioglioma with a small-cell glioblastoma component.

非機能性膵内分泌腫瘍の１切除例

We report a rare case of a very large nonfunctioning endocrine tumor of the pancreas without malignant histological features. A 63-year-old woman referred for appetite loss and general fatigue was found to have a tumor in the pancreas head. Computed tomography demonstrated a well-defined pancreatic tumor 45mm in diameter with hypervascular staining in the pancreas head. Angiography showed a hypervascular tumor of the pancreas head and a dilatation of the anterior superior and posterior superior pancreaticoduodenal arteries. The preoperative diagnosis was an endocrine tumor of the pancreas, with undeniable malignancy. Pylorus-preserving pancreaticoduodenectomy was performed. The histopathological diagnosis was a benign nonfunctioning endocrine tumor of the pancreas based on immunohistochemical staining for Chromogranin A, Synaptophysin, and NSE, but not for hormones. The tumor revealed a low labeling index (<2.0%) of Ki-67 indicating its benign character. No tumor recurrence has been identified in the 18 months since surgery.

"Malignant" Uterine Perivascular Epithelioid Cell Tumor, Pelvic Lymph Node Lymphangioleiomyomatosis, and Gynecological Pecomatosis in a Patient With Tuberous Sclerosis

We report a case of uterine perivascular epithelioid cell tumor (PEComa) with malignant histological features in a 59-year-old woman with tuberous sclerosis (TBS). The patient also had extrapulmonary lymphangioleiomyomatosis involving pelvic lymph nodes, myometrium, cervix, and ovary ("pecomatosis"). The uterine tumor measured 2.6 cm and had marked nuclear pleomorphism, necrosis, and 2 mitoses per 50 high-powered field, with an occasional atypical mitosis and infiltrative borders. The nonneoplastic myometrium, the cervical wall, and the hilum of the ovary had multiple clusters of bland-looking epithelioid clear cells that ranged from 1 to 5 mm (pecomatosis). The uterine tumor cells were positive for HMB-45 (90%), Melan-A (70%), smooth muscle actin (50%), and estrogen receptor (30%). Of the 16 pelvic lymph nodes excised, 3 were involved with lymphangioleiomyomatosis that was positive for HMB-45 and estrogen receptor. This is only the second reported PEComa associated with pecomatosis and the fourth PEComa described in a patient with TBS. The clinical significance of pecomatosis is still uncertain but seems to be seen only in patients with TBS.

Solitary fibrous tumor of the liver expressing CD34 and vimentin: A case report

A case of a successfully treated solitary fibrous tumor (SFT) of the liver is reported. An 82-year-old female presented with left upper abdominal discomfort, a firm mass on palpation, and imaging studies revealed a large tumor, 15 cm in diameter, arising from the left lobe of the liver. A formal left hepatectomy was performed. Microscopic evaluation showed spindle and fibroblast-like cells within the collagenous stroma. Immunohistochemistry disclosed diffuse CD34 and positive vimentin, supporting the diagnosis of a benign SFT. The patient remained well 21 months after surgery. SFT of the liver is a very rare neoplasm of mesenchymal origin. In most cases it is a benign lesion, although some may have malignant histological features and recur locally or metastasize. With less than 30 reported cases in the literature, little can be said regarding its natural history or the benefits of adjuvant radiochemotherapy. Complete surgical resection remains the cornerstone of its treatment.

Ameloblastic carcinoma of the maxilla: case report and review of the literature

A case of ameloblastic carcinoma of the maxilla arising in a 90-year-old patient is presented along with a review of 65 other cases of the international literature. The median age was 44 years with a predominance of men (42/66). The maxilla was concerned in almost one third of cases (21/66). Twenty patients died of disease after a median time of 60 months. Fifteen patients died with metastatic spread in the lung, brain, or bones; the others were due to a local recurrence. The specific survival rate was 68.7% at 5 years. Ameloblastic carcinoma is a rare entity of odontogenic tumors that exhibits malignant histologic features in the primary site. Specific mortality, estimated at 31.3% at 5 years, was generally due to a metastatic spread.

Diffuse leptomeningeal seeding from benign choroid plexus papilloma

Choroid plexus papillomas (CPP) are rare intracranial tumours with a favourable long-term outcome after surgical excision. Although they are histologically benign, local recurrences may occasionally occur, but leptomeningeal dissemination is exceptional. We report an unusual example of a fourth ventricle choroid plexus papilloma with diffuse leptomeningeal seeding. Neither the initial tumour nor the recurrence showed malignant histological features. Treatment with systemic and intrathecal chemotherapy was ineffective in this patient. We review the literature concerning leptomeningeal dissemination of benign choroid plexus papillomas.