PubMed Health⌕ Search

Biomedical subjects

William B Laskin

Publications and source records attributed to William B Laskin.

At least 19 recordsLinked to original sources
PubMed2007

Calcareous lesions of the distal extremities resembling tumoral calcinosis (tumoral calcinosislike lesions): clinicopathologic study of 43 cases emphasizing a pathogenesis-based approach to classification.

Herein, we analyze the clinicopathologic features of 46 distal extremity lesions that have histologic features similar to conventional tumoral calcinosis (tumoral calcinosislike; TC-L). The study included 31 females and 12 males (whites:non-whites>3:1) ranging in age from 1 to 91 (mean, 39; median, 42) years. The lesions presented in fingers (n=20), feet (n=10), wrist (n=6), hands (5), toes (n=4), and ankle (n=1) were solitary in all but 5 patients and ranged in size from 0.3 to 4.5 (mean, 1.6; median, 1.4) cm. Chief initial complaints included presence of a painful (n=16) or asymptomatic (n=7) mass, and limitation of joint mobility (n=3). Pertinent clinical associations included antecedent trauma (n=7), scleroderma (n=3), long-standing osteoarthritis (n=3), bony deformities (n=5), including 2 infants with congenital hand malformations, and chronic renal failure (n=2). Patients were stratified into one of the 3 recognized clinical settings of TC: primary normophosphatemic (n=17), secondary (n=5), or primary hyperphosphatemic TC (n=1). The 20 remaining patients were placed in an "indeterminate TC" category. Most lesions were located in tenosynovial/fascial tissue, but 13 lesions involved dermis and 1 was intra-articular. Histologically, the process consisted of multiple cystic or cleftlike spaces bordered by histiocytes, osteoclastlike giant cells, and a variable inflammatory infiltrate and containing fibrin, granular calcific debris, and calcospherites. Pools of calcific debris bordered by sclerotic collagen and a sparse cellular element predominated in 4 cases. Cartilaginous metaplasia was identified in 10 lesions and evidence of hemorrhage or specific injury was observed in 12 examples. Follow-up data for 22 patients (interval range, 1 to 30 y; median, 6 y) revealed 17 individuals with no evidence of recurrent disease or the development of additional lesions after simple (local) excision. One patient (indeterminate TC) required reexcision of a thumb mass 1 year after surgery. All 3 scleroderma patients developed additional TC-L lesions. Acral TC-L lesions are histologically similar to conventional TC, but present as smaller size lesions. Most TC-L lesions are closely aligned with primary normophosphatemic or secondary TC. Acral TC-L lesions may be the first manifestation of scleroderma, where the process has the potential to follow an unrelenting course.

Adolescent↗
PubMed2006

Postradiation angiosarcoma of the small intestine: a case report and review of literature.

Postradiation angiosarcoma is typically a high-grade sarcoma that presents mainly in the skin and superficial tissues. Postradiation angiosarcoma arising in the small intestine is rare with only 11 cases documented in the English-language literature. Herein, we report a postradiation angiosarcoma of the small intestine 9 years after radiotherapy for uterine cervical adenocarcinoma. The patient presented with symptoms of intestinal obstruction. At exploratory laparotomy, tumor nodules involved the small bowel. Microscopically, the neoplasm was composed of spindled and epithelioid cells arranged in solid aggregates and focally forming vascular channels. The diagnosis of angiosarcoma was confirmed immunohistochemically by tumor cell expression of CD31, CD34, and factor VIII-related antigen. The patient died 10 months after laparotomy. The diagnosis of PRA should be entertained for any poorly differentiated neoplasm arising in a previously irradiated site. The correct diagnosis of PRA depends upon histomorphologic identification of vascular differentiation, coupled with immunohistochemical expression of endothelial-related markers.

Adenocarcinoma↗
PubMed2006

Prognostic value of DNA ploidy, bcl-2 and p53 in localized prostate adenocarcinoma incidentally discovered at transurethral prostatectomy.

PURPOSE: Discovery of prostatic adenocarcinoma limited to transurethral resection material generates a treatment dilemma. We investigated the usefulness of parameters shown to be associated with prognosis in prostate cancer (p53 and bcl-2 immuno-expression, DNA cell cycle analysis and Gleason score) to stratify these incidentally identified tumors to guide clinical decision making. MATERIALS AND METHODS: Paraffin embedded tissues from transurethral prostate resection specimens containing T1a prostate adenocarcinoma from 44 patients who underwent resection between 1980 and 1990 were immunostained for p53 and bcl-2, and subjected to flow cytometry to determine DNA ploidy. Gleason score was determined by 2 pathologists independently. Statistical relationships among these 4 variables, tumor progression and cancer specific survival were analyzed. RESULTS: Six of 44 patients in the study population had cancer progression. Time to clinical progression was 4.5 years (range 7 months to 11 years). Most tumors stained negative for p53 and bcl-2. Only 2 tumors studied were aneuploid and neither of these 2 patients had cancer progression. Only Gleason score was a significant predictor of cancer progression on univariate and multivariate Cox regression analysis (p = 0.045 and 0.046, respectively). No tumor characteristics correlated with time to disease progression, including p53 and bcl-2 immuno-expression, and Gleason score (p = 0.182, 0.563 and 0.346, respectively). Positive immunostaining for p53 and bcl-2 did not occur together in the same tumor in significant fashion (p = 0.334), nor did either significantly occur more with aneuploidy (p = 0.237 and 0.307 respectively). CONCLUSIONS: For T1a prostate cancer incidentally detected on transurethral prostate resection p53 and bcl-2 immuno-expression, and DNA ploidy do not predict survival or disease progression.

Adenocarcinoma↗
PubMed2006

Sclerotic (fibroma-like) lipoma: a distinctive lipoma variant with a predilection for the distal extremities.

This report details the clinicopathologic and immunohistochemical findings identified in 21 cases of a fibrosclerotic variant of lipoma from acral sites that is frequently misdiagnosed as a fibromatous (nonlipogenic) process. The study includes 12 males and 9 females; aged 7 to 72 (mean and median, 39). The patients presented with solitary, mostly asymptomatic, masses that ranged from 0.6 to 2.2 (median, 1.2; mean, 1.3) cm and involved fingers (n=17), hands or wrists (n=3), and toes (n=1). Microscopically, the lesions were well-circumscribed nodules that showed very low to moderately low cellularity and consisted of cytologically bland spindled and stellate-shaped cells and a minor component of randomly dispersed adipocytes embedded in a collagenous to myxocollagenous stroma. Eight tumors were "fibroma-like" owing to their paucity of spindled cells, dense fibrosclerotic stroma, and inconspicuous vasculature. One tumor showed features of spindle cell/pleomorphic lipoma, whereas another demonstrated a vague onion skin-like arrangement of collagen reminiscent of sclerotic (storiform) fibroma. In all cases, the mitotic activity was negligible. Nonlipogenic tumor cells were immunoreactive for CD99 (6 of 6 cases), CD34 (6 of 8), S-100 protein (4 of 7), and smooth muscle actin (2 of 6). Follow-up data on 8 patients (range, 1 to 20 years; median, 9.5 years) revealed no recurrence in 6, but indicated the possibility of persistent tumor in the remaining 2 individuals after simple excision. Despite histological overlap in 1 case with the sclerotic fibroma, no patient displayed definitive clinical features of Cowden syndrome. Our study indicates that fibrosclerotic lipomas demonstrate a broader histological scope than what was initially described.

Adolescent↗
PubMed2005

Capillary hemangioblastoma of soft tissue: report of a case and review of the literature.

Capillary hemangioblastoma (CH) is a tumor of unknown histogenesis that arises primarily in the posterior cranial fossa, either as a sporadic event or in association with von Hippel-Lindau disease. To date, only 6 examples of a tumor with morphological features of CH arising in the somatic soft tissues have been documented in case reports and small series, and 3 of these tumors were associated with a peripheral nerve. Herein, we report a case of CH arising in the gastrocnemius muscle and not associated with a peripheral nerve in a 53-year-old woman with no clinical stigmata or family history of von Hippel-Lindau disease.

Antigens, CD34↗
PubMed2005

Villonodular synovitis (PVNS) of the spine.

OBJECTIVE: To describe the imaging features of spinal pigmented villonodular synovitis (PVNS). DESIGN AND PATIENTS: We retrospectively reviewed 15 cases of pathologically proven spinal PVNS. Patient demographics and clinical presentation were reviewed. Radiologic studies were evaluated by consensus of two musculoskeletal radiologists for spinal location, spinal segments affected, lesion center, detection of facet origin and intrinsic characteristics on radiography (n=11), myelography (n=7), CT (n=6) and MR imaging (n=6). RESULTS: Women (64%) were more commonly affected than men (36%) with an average age of 28 years. Clinical symptoms were pain (45%), neurologic (9%) or both (36%). Lesions most frequently affected the cervical spine (53%) followed by the thoracic (27%) and lumbar regions (20%). The majority of lesions (93%) were centered in the posterior elements with frequent involvement of the pedicle (67%), neural foramina (73%), lamina (67%) and facets (93%). No lesions showed calcification. Determination of a facet origin by imaging was dependent on imaging modality and lesion size. A facet origin could be determined in 45% of cases by radiography vs 67% of patients by CT (n=6) and MR (n=6). Large lesions (greater than 3 cm in at least one dimension) obscured the facet origin in all cases with CT and/or MR imaging (44%,n=4). Small lesions (less than 3 cm in any dimension) demonstrated an obvious facet origin in all cases by CT and/or MR imaging (56%,n=5). Low-to-intermediate signal intensity was seen in all cases on T2-weighted MR images resulting from hemosiderin deposition with "blooming effect" in one case with gradient echo MR images. CONCLUSIONS: PVNS of the spine is rare. Large lesions obscure the facet origin and simulate an aggressive intraosseous neoplasm. Patient age, a solitary noncystic lesion centered in the posterior elements, lack of mineralization and low-to-intermediate signal intensity on all MR pulse sequences may suggest the diagnosis in these cases. Small lesions demonstrate a facet origin on CT or MR imaging. This limits differential considerations to synovial-based lesions and additional features of a solitary focus, lack of underlying disease or systemic arthropathy, no calcification as well as low-to-intermediate signal intensity on all MR images should allow spinal PVNS to be suggested as the likely diagnosis.

Adolescent↗
PubMed2005

Granular cell tumor of the penis: clinicopathologic evaluation of 9 cases.

The occurrence of granular cell tumor (GCT) in penile tissue is very rare, with only 9 examples reported to date in the English-language literature. Herein, we describe the clinicopathologic and immunohistochemical findings in 9 additional cases. The patients ranged in age from 20 to 60 years (mean, 42 years; median, 40 years) at time of diagnosis. All penile tumors were solitary and arose in the dermis of the penile shaft (n=4), prepuce (n=3), and corona (n=2). A patient had a history of multiple cutaneous GCTs. Duration of symptoms before surgery ranged from 5 days to 2 years with the presence of an asymptomatic nodule representing the most common tumor-related complaint (n=8). The lesions ranged in size from 0.6 to 2.5 cm (mean, 1.5 cm; median, 1.5 cm). Microscopically, the tumors were moderate to highly cellular and were composed of oval to polygonal-shaped cells with abundant coarsely granular eosinophilic cytoplasm. Tumor cells grew in infiltrating nests, cords, and trabeculae and showed neural (n=2) and vessel wall (n=1) invasion or formed a relatively well-marginated solid nodule. Bland cytological features with only rare cells showing nucleomegaly (n=7) or spindling (n=3) were exhibited by 8 tumors. A tumor demonstrated diffuse nuclear atypia and was classified as "atypical." Mitotic activity ranged from 0 to 8 mitoses (mean, 1.4 mitoses) per 50 high-powered fields with no atypical division figures identified. All tumors tested showed moderate to strong immunohistochemical expression of S100 protein (n=6) and low-affinity nerve growth factor receptor (n=5), which was useful for detecting small deposits of tumor and helpful in evaluating surgical margins. Focal tumor cell immunoreactivity was observed for calretinin (4/6 cases) and glial fibrillary acidic protein (1/6 cases). All patients underwent simple (local) excision of their tumor. Complete follow-up data (mean, 21 years; interval range, 0.5-28 years) were available for 6 patients. No patient experienced recurrence or metastatic spread of tumor although surgical margins were microscopically involved by tumor in 5 cases. Benign GCT involving superficial soft tissue of the penis can be adequately managed by a simple excision. Patients with microscopically involved surgical margins can be clinically followed without immediate additional surgery.

Adult↗
PubMed2005

C-kit expression in renal oncocytomas and chromophobe renal cell carcinomas.

C- kit encodes the membrane-bound tyrosine kinase KIT, whose expression has been identified in several types of human neoplasms. Recently, KIT has been reported to be a marker for chromophobe renal cell carcinoma (RCC) and renal angiomyolipoma. However, expression of this molecule has not been adequately studied in other renal tumors, particularly oncocytoma, which may morphologically resemble chromophobe RCC. In this study, we analyzed c- kit messenger RNA (mRNA) levels in 17 chromophobe RCCs and 20 renal oncocytomas obtained from complementary DNA (cDNA) microarrays. Furthermore, comprehensive immunohistochemical analysis of KIT protein using a monoclonal antibody was performed in 226 renal tumors including chromophobe RCC (n=40), oncocytoma (n=41), clear-cell RCC (n=40), renal angiomyolipoma (n=29), and papillary RCC (n=21) on tissue microarrays (TMAs) and was compared with immunostaining results from 25 chromophobe RCCs and 30 oncocytomas using standard sections. The staining intensity was semiquantitatively graded on a 3-tier scoring system. All chromophobe RCCs and oncocytomas showed significant overexpression of c- kit mRNA. The average increase of mRNA compared with normal kidney tissue was 7.4-fold for chromophobe RCCs and 7.4-fold for oncocytomas. Immunohistochemical expression of KIT was found in most chromophobe RCCs (95% in TMAs and 96% in conventional sections) and oncocytomas (88% in TMAs and 100% in conventional sections) but was infrequently observed in renal angiomyolipomas (17%), papillary RCCs (5%), and clear-cell RCCs (3%). Furthermore, the average KIT immunoreactivity in TMAs was stronger in chromophobe RCC (1.93) and oncocytoma (2.07) than in other subtypes of renal tumors tested, including angiomyolipomas (0.17), papillary RCCs (0.05), and clear-cell RCCs (0.03). In conclusion, we found a significant elevation of c- kit mRNA by cDNA expression microarrays and overexpression of KIT protein by immunohistochemistry not only in chromophobe RCCs but also in oncocytomas. In contrast, immunohistochemical expression of KIT was not detected in most other types of renal cell tumors evaluated. The differential expression of c- kit in these renal tumors may have diagnostic and therapeutic implications.

Adenoma, Oxyphilic↗
PubMed2005

Benign epithelioid peripheral nerve sheath tumors of the soft tissues: clinicopathologic spectrum of 33 cases.

Benign epithelioid peripheral nerve sheath tumors (BEPNSTs) have not been fully characterized, and their relationship to conventional schwannoma and neurofibroma has not been satisfactorily established. Herein, we detail the clinicopathologic features of 33 examples of BEPNST. The study included 22 females and 11 males ranging in age from 2 to 68 years (median, 31.5 years). Only one patient probably has neurofibromatosis type 1. The tumors were predominantly dermal/subcutaneous in location (85%) and involved the lower limb (n=15), upper limb (n=11), trunk (n=4), and head/neck (n=3). The lesions ranged in size from 0.3 to 6.8 cm (median, 1.1 cm). Microscopically, the tumors were generally well-circumscribed, uninodular, or multinodular masses. Twenty-six lesions were encapsulated. Tumors consisted of trabeculae, loosely arranged nodules, and cohesive nests of epithelioid tumor cells immersed in collagenous, myxohyaline, or chiefly myxoid stroma. A bland spindled cell component comprising 5% to 40% of the tumor was noted in 15 cases. Mitotic activity ranged from 0 to 6 mitoses/50 high power fields (mean, 1.5 mitoses/50 high power fields) with no abnormal division figures identified. Five lesions were considered atypical based on presence of focal nuclear/nucleolar enlargement and hyperchromasia. Immunohistochemical reactivity for Schwann cell-related markers in tumor cells included S-100 protein (20 of 20 cases), collagen type IV (10 of 10), laminin (8 of 8), nerve growth factor receptor, p75(7 of 8), CD57 (6 of 9), and glial fibrillary acidic protein (8 of 15). CD34-positive fibroblast-like cells were identified in all 12 neoplasms tested. Anti-epithelial membrane antigen highlighted perineurial cells in 9 of the 11 encapsulated tumors. Anti-neurofilament protein did not identify intralesional neuraxons in the 10 tumors evaluated. Eighteen tumors were subtyped as epithelioid neurofibromas. The remaining 15 cases showed some histologic features suggestive of schwannoma, but their uniform cellularity, absence of nuclear palisading, and presence of a significant CD34-positive spindled cell population in 5 cases led to their classification as "BEPNST of indeterminate histogenesis." Evaluation for loss of heterozygosity in 2 cases demonstrated deletion of genetic material on chromosome 22q and 17q involving NF2 and NF1 loci. However, sequencing of NF2 coding sequences revealed no mutations. Follow-up for 18 patients (median interval, 13.5 years), including 4 patients with tumors exhibiting cytologic atypia, revealed a nondestructive recurrence or persistent disease in 3 patients whose tumors lacked atypia, but no evidence of metastatic spread or tumor-related death. BEPNSTs are usually small neoplasms located in superficial soft tissue and have an excellent prognosis after complete local excision. Accurate subclassification of some of these lesions is difficult based on currently available techniques.

Adolescent↗
PubMed2005

Nerve sheath myxoma: a clinicopathologic and immunohistochemical analysis of 57 morphologically distinctive, S-100 protein- and GFAP-positive, myxoid peripheral nerve sheath tumors with a predilection for the extremities and a high local recurrence rate.

This report describes the clinicopathologic findings in 57 cases of nerve sheath myxoma. Our study group included 34 males and 23 females, ranging from 8 to 72 years of age at the time of their first surgical procedure (mean, 36 years; median, 34 years). The patients typically presented with solitary, superficial, multinodular masses in the 0.5- to 2.5-cm size range. Eighty-six percent of cases occurred in the extremities, with the most common locations being the hand/fingers (n = 22), knee/pretibial region (n = 10), and ankle/foot (n = 7). Only 7 cases (12.3%) involved the trunk or head and neck region. The tumors were generally slow growing, and often, they were present for many years before surgical resection was sought. In the majority of instances, the lesions were painless. Histologically, the tumors involved the dermis and/or subcutis, and they formed distinct multinodular/multilobular masses with abundant myxoid matrix and a peripheral fibrous border. All cases had small epithelioid Schwann cells in corded, nested, and/or syncytial-like aggregates, a variable number of Schwann cells with a ring-like appearance, and scattered spindled and stellate-shaped Schwann cells. These cells often had cytoplasmic-nuclear invaginations, and they were immunoreactive for S-100 protein, glial fibrillary acidic protein, neuron specific enolase, and CD57. They were also bordered by collagen IV. Epithelial membrane antigen-positive perineurial cells were typically present in small numbers, primarily in the fibrous tissue directly adjacent to the myxoid nodules. CD34-positive intraneural fibroblasts were generally sparse. Mitotic figures were uncommon. All cases were initially managed by simple excision, and in almost all instances, tumor extended to the tissue edge. Follow-up information is available for 34 patients (follow-up range, 8 months to 28 years; median follow-up interval, 14 years 3 months). Sixteen patients (47%) had one (n = 11) or more (n = 5) local recurrence of their tumor, and 2 additional patients had findings suspicious for a recurrence. Nerve sheath myxomas are morphologically distinct peripheral nerve sheath tumors with a peak incidence in the fourth decade of life and a strong predilection for the extremities. These tumors have a relatively high local recurrence rate when managed by simple local excision. They appear to be unrelated to so-called cellular and mixed-type neurothekeomas.

Adolescent↗
PubMed2005

Lymphangioma-like Kaposi sarcoma.

BACKGROUND: Lymphangioma-like Kaposi's sarcoma (LLKS) is a rare morphologic expression of Kaposi's sarcoma (KS) that occurs in virtually all of the well-recognized clinical subtypes of the disease and has the potential to mimic other pathologic processes. In this study, we present the clinical and pathological features of four patients with LLKS. METHODS: Four cases of LLKS were retrieved from the dermatopathology files of our institution. All four tumours were tested immunohistochemically with anti-human herpesvirus-8 (HHV-8) latent nuclear antigen-1 (LNA-1) and anti-CD34 antibodies. RESULTS: Clinically, each patient presented with violaceous patches, papules or plaques; one patient presented with bullous lesions. All of the LLKS biopsy specimens revealed areas with characteristic light microscopic features of KS. Lymphangioma-like foci consisted of ectatic, irregularly shaped vascular spaces lined by mildly atypical endothelial cells. All tumour cells, including those associated with LLKS foci, showed a strong and diffuse reactivity for anti-HHV-8 LNA-1 and anti-CD34. KS progressed slowly in two patients with adequate follow-up. CONCLUSIONS: As LLKS can mimic other disease processes, the correct diagnosis relies heavily on the recognition of salient clinical and histological features of conventional KS, including a strong immunohistochemical expression of HHV-8-associated LNA-1 in lesional cells.

Adult↗
PubMed2005

Palmar-plantar fibromatosis in children and preadolescents: a clinicopathologic study of 56 cases with newly recognized demographics and extended follow-up information.

Palmar-plantar fibromatosis, the most common type of fibromatosis, is well recognized in the adult population, but many clinicians and pathologists are unfamiliar with the fact that children may also be affected by this process. This report describes the clinicopathologic findings in 56 cases of palmar-plantar fibromatosis in children and preadolescents. Our study group included 19 males and 37 females, ranging from 2 to 12 years of age at the time of their first surgical procedure (median age, 9 years). The patients typically presented with solitary, lobular or multilobular masses in the 0.5- to 2.5-cm size range. The preoperative duration of the lesions ranged from 1 month to 6 years, with 1 patient purportedly having clinical evidence of disease since birth. All but two of the initial lesions occurred on the plantar aspect of the feet, typically in the region of the arch. Only 2 patients presented with palmar disease. The tumors were usually painless, except when pressure was applied. Seven patients had a history of trauma, sometimes involving a foreign body. One patient presented with concurrent disease involving both feet, and 12 additional patients subsequently developed palmar-plantar fibromatosis in another extremity, knuckle pads on the hands, or had other clinical findings linked to this disease. A family history was available for 25 patients, and 11 individuals had relatives with palmar-plantar fibromatosis, and 4 others had relatives with a history that was either suspicious for palmar-plantar disease or positive for other disorders associated with this disease. Histologically, the tumors involved aponeurosis and commonly formed discontinuous, moderately cellular, nodular masses composed of spindled cells with intervening collagen. Mitotic counts for 79 separately submitted tumor specimens ranged from 0 to 31 mitotic figures per 25 wide-field high power fields (mean mitotic count, 3.4 mitotic figures per 25 wide-field high power fields). Eight tumor had > or =10 mitoses per 25 wide-field high power fields. All patients were initially managed by local excision, and in most of cases, histologic examination showed tumor extending to the tissue edge. Thirty-two of 38 patients (84.2%) with clinical follow-up, ranging from 4 months to 33 years (mean, 14 years 9 months; median, 16 years 1 month), had one (n = 16) or more (n = 16) local recurrence of their fibromatosis.

Child↗
PubMed2004

Multifocal presentation of gangliocytic paraganglioma in the mediastinum and esophagus.

Gangliocytic paraganglioma (GP) is a rare, typically benign tumor that shows neuroectodermal (neurosustentacular or Schwannian and neuronal) and neuroendocrine differentiation. Once thought to arise exclusively in the periampullary region as a solitary lesion, recent reports have documented both origin of GP in a variety of extra-duodenal sites as well as synchronous multifocal presentation of the tumor. Herein, we describe the first reported case of simultaneous occurrence of GP in the superior mediastinum and esophagus. A mass in the mid-distal esophagus and a separate mass in the superior mediastinum at the thoracic inlet were found in a 58-year-old woman by computed tomography scan. Subsequent biopsy of the superior mediastinal mass showed nests of epithelioid tumor cells coexisting with ganglioneuromatous elements, whereas biopsy of the esophageal mass showed nests of epithelioid cells with interspersed ganglion cells. The epithelioid tumor cells showed diffuse immunohistochemical expression of keratin (CAM 5.2), chromogranin, and synaptophysin supporting true neuroendocrine differentiation; ganglion cells expressed S-100 protein and neurofilament protein; and the spindled elements expressed S-100 protein, neurofilament protein, and glial fibrillary acidic protein indicating Schwannian differentiation. The finding of another GP occurring outside the periampullary region bolsters the argument for a stem cell origin of this unusual tumor.

Esophageal Neoplasms↗
PubMed2004

Intracranial chondrosarcoma: a case report and review of the literature.

OBJECTIVE AND IMPORTANCE: Chondrosarcomas rarely occur intracranially away from the skull base. We present a case of a supratentorial dural-based, low-grade classic chondrosarcoma and emphasize salient imaging findings that assist in its diagnosis. CLINICAL PRESENTATION: A 23-year-old female presented with new onset seizures. Multiple radiographic imaging studies identified a large left frontal mass with radiological findings that suggested the correct diagnosis. INTERVENTION: The patient underwent a left frontal craniotomy and gross total resection of the lesion and associated dura. CONCLUSION: Intracranial chondrosarcomas away from the skull based are extremely rare malignancies. As with other extra-axial masses, they often grow to a relatively large size before generating symptoms. Careful analysis of CT, MR, and angiographic imaging characteristics should suggest the correct diagnosis.

Adult↗
PubMed2004

Ectopic hamartomatous thymoma: a clinicopathologic and immunohistochemical analysis of 21 cases with data supporting reclassification as a branchial anlage mixed tumor.

This report describes the clinicopathologic and immunohistochemical findings in 21 cases of a highly distinctive tumor with a strong predilection for the lower neck region of adult males. Our study group consisted of 20 males and one female. The patients were 28 to 79 years old (mean age, 47 years; median age, 40 years), and they presented with solitary, lobular or multilobular masses ranging in size from 2.0 to 19.0 cm in greatest dimension (mean size, 5.1 cm; median, 4 cm). The tumors principally involved the lower neck region, usually in close proximity to the sternoclavicular joint. The preoperative duration of the lesions ranged from 2 months to 30 years. Histologically, the tumors were typically well marginated and composed of plump spindled cells, delicate spindled cells, mature adipose tissue, and epithelial cells, including both squamous and glandular elements. Epithelial-lined cysts were a focal finding in most cases and measured up to 2 cm in greatest dimension. Mitotic counts for the tumors ranged from 0 to 7 mitotic figures per 50 high power fields (mean mitotic count, 1.1 mitotic figures per 50 HPFs). Our immunohistochemical analysis revealed a complex immunophenotype with a diverse keratin profile. The plump spindled cells had a myoepithelial phenotype, as evidenced by the coexpression of keratins (5, 5/6, and 14), alpha-smooth muscle actin, CD10, and to a lesser extent, calponin. No compelling evidence for thymic differentiation was observed. The patients were initially managed by biopsy or partial resection (n = 4), simple local excision (n = 16), or an unspecified procedure (n = 1). Clinical follow-up of > or =3 years was available for 10 patients (48%). Two patients had recurrent disease, but there were no metastases or tumor-related deaths. A derivation from sequestered branchial epithelium is likely, but evidence for a thymic component is tenuous, at best. Our data support reclassification of this distinctive process as a branchial anlage mixed tumor. The differential diagnosis includes conventional mixed tumors of skin adnexal or salivary gland origin, synovial sarcoma, a peripheral nerve sheath tumor variant, and cystic teratoma.

Adult↗