Significant Pleomorphism Research Articles

Proliferating inverted follicular keratosis of genital skin: Case series of a challenging variant.

Inverted follicular keratosis (IFK) is a benign cutaneous epithelial tumor typically presenting as a small papule on the head and neck. We have encountered deep endophytic tumors on genital skin with some characteristics of IFK but also atypical features, raising concern for squamous cell carcinoma (SCC). Four such tumors were identified in our database. Histopathologic analysis and ancillary studies were performed. All patients were young women who presented with a solitary 0.5-1.0 cm lesion on the vulva, perineum, or inner buttock. Each showed a squamous proliferation arising from the epidermis, with endophytic growth that was deep and bulbous but not infiltrative. The tumor lobules contained eosinophilic keratinocytes, forming numerous squamous eddies. Small irregular spaces and dyskeratotic cells were frequently found. Nuclear pleomorphism was minimal to absent. All demonstrated wild-type p53 expression and lack of p16 block positivity. In situ hybridizations for human papillomavirus were negative. None of the three cases with follow-up data showed evidence of recurrence. The absence of infiltrative growth or significant pleomorphism, the presence of numerous squamous eddies, the reassuring immunoprofile, and the lack of evidence of recurrence support a variant of IFK and speak against SCC. We propose the term "proliferating IFK" to highlight the florid squamous proliferation. Recognition of this unusual variant would avoid overdiagnosis of SCC.

Primary leiomyosarcoma of the bone: a case report.

Primary leiomyosarcoma of the bone is rare. Histologically, it resembles leiomyosarcoma of soft tissue. Given the rarity of this entity, its diagnosis should be made only after clinical studies and workup have excluded metastasis from other sites. Herein, we describe an additional case of primary bone leiomyosarcoma. We report a 32-year-old female patient, who presented with right knee pain and was found to have a right distal femur mass by imaging studies. Biopsy showed a neoplasm composed of fascicles of spindle cells, arranged in different patterns, with significant pleomorphism. The tumor cells were positive for smooth muscle actin, focally positive for desmin and H-caldesmon. No other masses in the body were detected by imaging studies. The diagnosis of leiomyosarcoma of the bone was rendered. Given the broad diagnostic differential of primary bone leiomyosarcoma, it is important to be aware of this rare bone tumor phenotype and of its histomorphologic and immunohistochemical features for an accurate diagnosis.

Pediatric Pleomorphic Xanthoastrocytoma with Neoplastic Meningitis: A Case Report with Cytopathological Evidence with Literature Review

AbstractPleomorphic xanthoastrocytoma (PXA) was regarded as grade II tumor and considered to be associated with favorable outcome. The World Health Organization Central Nervous System 5 (WHO CNS5) has classified PXA under circumscribed astrocytic gliomas and graded 2 or 3 depending on histology. Cerebrospinal fluid (CSF) and leptomeningeal spread are observed rarely in these tumors. The present case report describes a PXA, grade 3 tumor in a young male with neoplastic meningitis. This 17-year-old male child presented with history of seizure, signs of raised intracranial pressure, and meningeal irritation. Well-defined, T2 heterogeneously hyperintense lesion (5.5*4.8 cm) was seen in right frontal lobe with mild heterogenous contrast enhancement and adjacent pachy-meningeal enhancement. Right frontal craniotomy and near total excision were done. Postoperative hydrocephalus was treated with CSF diversion. Histopathology showed epithelioid and rhabdoid morphology with significant cellular pleomorphism and atypical mitosis consistent with the PXA, grade 3. The CSF cytology showed numerous tumor cells with marked nuclear and cytoplasmic pleomorphism. PXA is a rare malignancy of children and young adults, commonly seen in the temporal lobes. BRAF point mutations of V600E type are most common in PXA, grade 2. Meningeal dissemination is uncommon in PXA and its presence marks poor outcome. PXA, grade 2 tumors could be followed with serial imaging following gross total resection. PXA, grade 3 tumors are managed with maximal-safe resection, radiotherapy, and/ or chemotherapy. PXA, grade 3 with CSF spread tends to have rapid decline in the clinical course and it is advisable to get routine baseline and follow-up craniospinal screening and needs aggressive management.

OSTEOSARCOMA OF THE MANDIBLE: CASE REPORT WITH EMPHASIS ON DIAGNOSIS AND TREATMENT

The osteosarcoma that affects the jaw bones consists of a rare malignant neoplasm that has the capacity to produce immature bone or osteoid. A 60-year-old male patient attended a referral service exhibiting a rapidly growing lesion with nodular appearance, mucosa-like staining, hardened consistency, and painful symptoms located in the right mandibular body. The radiographic examination showed a mixed aspect of radiolucency and radiopacity, exhibiting the classic pattern of sun rays. The lesion was submitted to incisional biopsy, and the histopathologic examination revealed a malignant neoplasm of bone origin characterized by a proliferation of mesenchymal cells with significant pleomorphism and an intense deposition of irregular osteoid matrix. After the diagnosis of osteosarcoma was established, the patient underwent complete surgical resection of the lesion and is currently being followed up after 1 year without clinical-radiographic signs of relapse.

Juxtaglomerular Cell Tumor With Atypical Pathological Features: Report of a Case and Review of Literature

Juxtaglomerular cell tumor (JGCT) is a rare renal tumor with a predominantly benign clinical course. It affects young adults, who often present with hypertension, hypokalemia, and hyperaldosteronism. The tumor cells are round to spindle-shaped with occasional mild to moderate atypia, but mitotic figures are usually absent. Surgical resection is the treatment of choice. Typically, the blood pressure and renin levels normalize after removal of the tumor. Rare cases of metastatic and recurrent JGCT have been reported including cases with vascular invasion. These cases typically occur in older adults and present with larger tumor size (9-15 cm). We report a case of JGCT, 5.5 cm in greatest dimension, with atypical pathological features including invasion of the renal vein, lymphovascular invasion, and significant pleomorphism with rhabdoid morphology, along with a brief review of the literature.

SMARCB1/INI1 Loss in Epithelioid Schwannoma

The epithelioid variant of schwannoma is rare, and loss of SMARCB1/INI1 expression has been observed in a subset of cases. Our aim was to further define the clinicopathologic features and to evaluate SMARCB1/INI1 deficiency in a large cohort of 65 epithelioid schwannomas diagnosed between 2002 and 2015, which consisted of 32 men and 33 women with median age at diagnosis of 45 years (range, 13 to 75 y). Most tumors arose in the extremities (upper, 20, lower, 15) and trunk (17); 9 were visceral (8 gastrointestinal). Most somatic tumors were in dermis/subcutis (53/54) and encapsulated (53/54) with an epithelial membrane antigen-positive perineurial capsule in 46 cases; visceral tumors were unencapsulated. No patients were reported to have any neurocristopathy. Three patients had multiple lesions (2 each). Tumor size range was 0.4 to 22.7 cm (median, 1.2 cm). Tumors showed multilobulated growth of uniform epithelioid cells in sheets and nests or singly dispersed within a frequently myxoid or hyalinized stroma. Tumor cells had round vesicular nuclei and abundant palely eosinophilic cytoplasm, usually lacking significant pleomorphism or hyperchromasia. Some tumors showed foci resembling conventional schwannoma (spindled morphology, 29; Antoni B foci or Verocay bodies, 8; hyalinized thick-walled vessels, 16). Mitoses ranged from 0 to 9 per 10 high-power fields (median count, 1). No tumor had necrosis. Twenty-three cases showed degenerative nuclear atypia. Focally striking cytologic atypia was present in 7 tumors, 3 of which showed transformation to epithelioid malignant peripheral nerve sheath tumor. All tumors showed diffuse positivity for S-100 protein and consistent positivity for SOX10 (50/50), while INI1 expression was lost in 24 of 57. Other positive immunohistochemical results were: glial fibrillary acidic protein (15/37) and focal keratin (2/40); epithelial membrane antigen (0/53) and melanocytic markers were negative (Mart-1 0/29; HMB-45 0/23). Most patients underwent local excision (13 complete; 47 marginal/positive margins). Follow-up data available for 31 patients (range, 1 to 108 mo; median, 37) indicated that no patient had developed metastatic disease, including 3 cases with cytologic atypia, one of which showed malignant transformation. One tumor without atypia developed local recurrence 48 months after marginal excision; all other patients were alive with no evidence of disease. Epithelioid schwannoma most commonly occurs as a superficial tumor on the extremities or trunk in adults. Loss of SMARCB1/INI1 expression is seen in 42% of tumors. Tumors follow a generally benign clinical course, although recurrence and malignant transformation are infrequent. Some tumors are characterized by notable cytologic atypia, the significance of which is uncertain but which may indicate a morphologic continuum with low-grade epithelioid malignant peripheral nerve sheath tumor.

Invasive Pleomorphic Lobular Histology is an Adverse Prognostic Factor on Survival in Patients with Breast Cancer

Invasive pleomorphic lobular carcinoma (IPLC) is defined to be an uncommon and different subtype of classical invasive lobular carcinoma (ILC). This special variant is characterized by significant cytological atypia and pleomorphism which differs from the cytological uniformity of ILC. IPLC has been shown to have some poor prognostic factors such as axillary node metastasis and higher histological grade which may lead to poor clinical courses including a short relapse time, increased risk of recurrence and a decreased survival. The aim of this study is to investigate the clinicopathological characteristics and prognosis of IPLC in comparison with ILC and also to evaluate if IPLC is a different clinical entity compared to ILC. A total number of 4418 breast cancer patients treated between 1996 and 2015 in Hacettepe University Cancer Institute, were retrospectively analyzed. Among 4418 patients, 210 were diagnosed with ILC and 23 patients diagnosed with pure IPLC. In this present study, clinicopathological characteristics, disease free survival (DFS) and overall survival (OS) of patients with ILC and IPLC were compared. This study design is one of the rare face to face comparison of pure IPLC and ILC. Patients with IPLC had an increased rate of higher histologic grade, extracapsular extension, lymphovascular invasion and lower percentage of hormone positivity than those of patients with ILC. During the follow-up time, IPLC group experienced 4 cases (17.3%) of recurrence, 5 cases (21.7%) of death and 2 cases (8.7%) of progression in 3 metastatic patients compared to that of 27 cases (12.9%) of recurrence, 29 cases (13,8%) of death and 14 cases (6.7%) of progression in 19 metastatic patients in the ILC group. Patients with IPLC had a worse DFS and OS duration than patients with ILC (P = 0.02 for OS, P = 0.04 for DFS). In conclusion, IPLC is a different and a special breast cancer subtype. This study suggests that IPLC is a distinct clinical entity with an advanced stage and more aggressive clinical course. Patients with IPLC reveal poorer prognostic factors such as higher histological grade, relative lower percentages of hormone positivity, and increased rate of recurrences resulting in poor clinical outcomes and worse survival. Nowadays, it is observed that current treatment methods for IPLC do not seem as successful as in ILC and failed to be effective. Thence, individual therapies including more aggressive surgery and adjuvant or neoadjuvant chemotherapy even in the earlier stages of breast cancer should be performed for this distinct variant.

Thick keratoconic cornea associated with posterior polymorphous corneal dystrophy

We herein report a case of bilateral unusually thick non-edematous keratoconic corneas with associated endothelial features of posterior polymorphous corneal dystrophy (PPCD). We report the case of a 27-year-old myopic woman who presented for refractive surgery. Slit lamp exam showed bilateral corneal protrusion with diffuse deep stromal and endothelial vesicular opacities and small paracentral bands. Topography showed generalized advanced corneal steepening in both eyes with increased anterior and posterior central corneal elevations in comparison to the best fit sphere. Ultrasound pachymetry showed central corneal thickness of 605μm (RE) and 612μm (LE). On specular biomicroscopy, cell density of 2503 cells/mm2 RE and 1526 cells/mm2 LE with significant cellular pleomorphism and polymegathism were noted. Clinical and paraclinical findings together suggest the presence of simultaneous keratoconus and PPCD. The literature has suggested an association between PPCD and steep cornea. Moreover, many reports have also described cases of associated PPCD and keratoconus with characteristic thinning and ectasia, in comparison to the unusual thick corneas noted in our patient, despite the absence of edema. Identification of genetics factors is further needed to clarify this association. This case describes a patient whose corneas present features of both keratoconus and PPCD and is unique due to the presence of increased corneal thickness despite the absence of edema.

A case of renal cell carcinoma with abundant smooth muscle (leiomyomatous) stroma and the differential diagnosis

Renal cell carcinoma with smooth muscle stroma (RCCSMS) is a rare and controversial renal cancer type with indolent behavior. This separation of this entity from other types of renal cell carcinoma is supported by recent published studies but not yet recognized by the World Health Organization. We present a case of a 72 years old female, with incidental finding on abdominal ultrasound of a renal nodule with 1.5cm. The patient underwent partial nephrectomy and the pathological evaluation showed an epithelial tumor composed of nests and trabeculae of polygonal cells with clear cytoplasm and low grade nuclear features, surrounded by an abundant stroma composed by fascicles of spindle cells without significant pleomorphism or mitoses. Immunohistochemically, the epithelial cells showed strong and diffuse positivity for CK7, CD10, vimentin and EMA; staining for RCC was focal. The stromal cells were positive for vimentin, smooth muscle actin, HHF35, desmin and caldesmon; ER and PR were negative. The staining for HMB45 and Melan-A was negative in both components. So the final diagnosis of RCCSMS was made. It’s important to be aware of this entity in order to differentiate RCCSMS from renal cancers with less favorable prognosis and higher degree of malignancy, like clear cell renal cell carcinoma, especially with sarcomatoid change.

Tumors with poroid features: a clinicopathologic analysis of 45 cases

To investigate the clinicopathological features and the differential diagnosis of poroma and porocarcinoma. Histopathological characteristics and clinical data of 35 cases of poroma and 10 cases of porocarcinoma were analyzed retrospectively. The average age of 35 patients of poroma was 48 years. The average age of 10 patients of porocarcinoma was 65 years. Both poroma and porocarcinoma occured most frequently on the scalp and face,as well as the extremities. Histologically, cases of poroma were divided into three subtypes, including classic poroma (23 cases), hidroacanthoma simplex (3 cases) and dermal duct tumor (9 cases). Residual foci of benign poroma were found in all cases of porocarcinoma, most of which were classic poroma. The malignant components showed severe dysplasia and/or stromal infiltration. The diagnosis of poroma and porocarcinoma is mainly based on the microscopic characteristics. An invasive architectural pattern and/or significant cytologic pleomorphism are the most important clues for the diagnosis of porocarcinoma. Neither focal mitotic activity nor the presence of necrosis was the diagnostic feature of porocarcinoma. Malignant transformation can occur in some cases of long existing poroma with recent, rapid tumor enlargement.

Clinicopathological analysis of solitary fibrous tumor in 11 patients

Objective To evaluate the clinicopathological characteristics and immunophenotype of solitary fibrous tumor (SFT), which is able to provide the basis for clinical diagnosis and prognosis, differential diagnosis and predictable prognosis. Methods Data from 11 cases of SFT were investigated by immunohistochemical. Results Histologically, SFT was composed of spindle cells, which arranged in fascicles pattern, as well as the collagen and branch vessels. Malignant SFT had higher and crowed cellularity with significant pleomorphism, numerous mitoses and putrescence. Positivity for bcl-2 and CD34 was observed in all SFT. Eight tissues showed CD99 positivity, one showed s-100 positivity, all 11 tissues showed Ckpan negativity. Conclusions SFT has originated from different sites, which should be distinguished with fibrosarcoma and other spindle cell neoplasms. More attentions should be paid to malignant SFT and focus on the follow-up visit. Key words: Solitary fibrous tumor; Immunohistopathology; Differential diagnosis

Extraskeletal chondrosarcoma of the chest wall mimicking as carcinoma of the breast-A case report

Extraskeletal myxoid chondrosarcoma (EMC) is a rare tumour and usually occurs in the soft tissue of the extremities. Problems can occur when the site is unusual and the cellular differentiation is not distinct. Chest wall is an unusual site for EMC. When aspiration cytology of breast mass yields polygonal cells with no significant pleomorphism in a prominent myxoid/mucoid background, one should take into consideration the possibility of EMC of the anterior chest wall before diagnosing it as breast carcinoma, as both the entities have different treatment modalities. We present a case in which EMC of the anterior chest wall presented as a breast mass in a 55-year-old female patient; it was misdiagnosed as mucinous/myxoid carcinoma of the breast on fine-needle aspiration cytology, and subsequently, correct diagnosis was made on histopathological examination. This case has been highlighted for the unusual site of occurrence of EMC and its close similarity to breast carcinoma, both clinically and cytologically.

A Rare Case of Intraoral Nodular Fasciitis: Diagnosis and Immunohistochemical Profile

Nodular fasciitis is a benign, idiopathic, reactive proliferation of myofibroblasts found in the subcutaneous fascia; intraoral occurrence is very rare. An 18-year-old woman was referred to the oral diagnosis service with a 1-month history of a nodular mass in the gingiva. Clinical examination disclosed a well-circumscribed, mobile, pedunculated mass in the left mandibular gingiva. The clinical diagnoses included pyogenic granuloma. She underwent an excisional biopsy under local anesthesia through an intraoral approach. Microscopic examination showed a proliferation of spindle cells arranged in intersecting fascicles. The spindle cells exhibited plump, vesicular nuclei without significant pleomorphism. Scattered multinucleated giant cells also were present. Immunohistochemical stains showed that the lesional cells were positive for smooth muscle actin and vimentin and negative for S-100 protein. The features were those of an inflammatory, benign myofibroblastic lesion, consistent with intraoral nodular fasciitis.

Metastatic Spiradenocarcinoma Occurring in an 8‐Year‐Old Boy

We recently saw the case of an 8-year-old boy with histologic findings of spiradenocarcinoma. Malignant adnexal tumors in children are exceedingly rare, and cases of spiradenocarcinoma in children are absent in the literature. We report the case of an 8-year-old boy with metastatic spiradenocarcinoma. A biopsy 2 years before his presentation at our institution was interpreted as a benign lesion, but when the lesion regrew, a repeat biopsy was performed that demonstrated ominous findings, prompting a reexcision. This reexcision demonstrated an area with significant necrosis, many mitoses, and cellular pleomorphism apparently arising out of a sharply demarcated, adjacent, lower-grade area. The histologic features of this patient's biopsies were those of spiradenocarcinoma, potentially arising out of a preexisting spiradenoma, a finding that has not been documented previously in this age group. Staging studies demonstrated multiple bilateral pulmonary nodules, which were confirmed using thoracoscopic biopsy to be metastatic disease. His chemotherapy regimen has included several cycles of cisplatin and 5-flourouracil, with ongoing disease progression of pulmonary disease. It has been 16 months since he presented to our institution (>3 years since the appearance of his initial lesion). Imaging studies demonstrate a slowly increasing size and number of pulmonary lesions. The long-term prognosis is guarded.

Fibroblastic Connective Tissue Nevus

Fibroblastic connective tissue nevus (FCTN) represents a rare and distinct benign cutaneous mesenchymal lesion of fibroblastic/myofibroblastic lineage, which broadens the spectrum of lesions presently recognized as connective tissue nevus. A series of 25 cases of FCTN has been analyzed to further characterize the clinicopathologic spectrum and immunohistochemical features of this entity. Sixteen patients were female (64%) and 9 were male (36%), with age at presentation ranging from 1.5 months to 58 years (median, 10 y). Most patients presented with a solitary, slowly growing, painless plaque-like or nodular skin lesion. Eleven cases (44%) arose on the trunk, 9 (36%) on the head and neck, and 5 (20%) on the limbs. The lesion was present for a median duration of 11.5 months (mean, 13.2 mo). Grossly, the lesions were tan-brown to tan-white, smooth, and firm. Their size ranged from 0.3 to 2.0 cm in greatest dimension (mean size, 0.67 cm; median, 0.6 cm). All tumors showed poor circumscription and were situated primarily in the reticular deep dermis, extending into the superficial subcutis in 13 cases (52%). The lesion was associated with papillomatous epidermis in 17 cases (70%) and the presence of adipose tissue in the reticular dermis in 14 cases (60.9%). All tumors were composed of a proliferation of bland intradermal fibroblastic/myofibroblastic cells with indistinct palely eosinophilic cytoplasm and tapering nuclei, with no significant cytologic atypia or pleomorphism, arranged in short-intersecting fascicles and entrapping appendages. No mitoses were identified. Immunostains showed positivity for CD34 in 20 of 23 cases (87%) and weak focal positivity for smooth muscle actin in 9 of 19 cases (47%). No case stained positively for desmin or S100 protein. Clinical follow-up was obtained for 14 patients (median duration, 4 y). No tumor recurred locally, even when surgical excision was incomplete. No lesion metastasized. FCTN occurs most commonly as a plaque on the trunk and head/neck of children, involves deep dermis and superficial subcutis, and stains mainly for CD34. FCTN most likely represents a localized developmental dermal anomaly; it is entirely benign and should not be confused with dermatofibrosarcoma protuberans or other neoplasms such as dermatomyofibroma.

Composite Intestinal Adenoma-Microcarcinoid

Composite intestinal adenoma and microcarcinoid is a rare intestinal neoplasm consisting of intermingled adenomatous and well-differentiated neuroendocrine components. A few case reports and small series have suggested an indolent clinical course for this entity. We reported 7 cases of composite intestinal adenoma-microcarcinoid, including their morphologic features and clinical follow-up, both in biopsy and resection specimens. We identified 7 cases of composite intestinal adenoma-microcarcinoid from our pathology database. Five were from the large intestine, and 2 were in the duodenum. Morphologically, all microcarcinoids exhibited low-grade cytologic atypia and were devoid of significant pleomorphism, necrosis, and mitotic activity. Among the 7 lesions, 6 had a lobular architecture with smooth borders and mucosa-confined microcarcinoids; none had neuroendocrine carcinoma in subsequent resections. However, 1 colonic case had carcinoid cells penetrating the muscularis mucosae into the submucosa with an infiltrative border, and the resection showed metastatic high-grade neuroendocrine carcinoma in 1 lymph node. Composite intestinal adenoma-microcarcinoid is extremely rare. Although composite mucosa-confined adenoma-microcarcinoid is likely to have an indolent behavior, submucosal invasion by the neuroendocrine component may be associated with aggressive behavior.

Hepatic epithelioid hemangioendothelioma in needle biopsy specimens: report of 5 cases with review of literature

To evaluate the pathologic diagnosis of hepatic epithelioid hemangioendothelioma (EH) in needle biopsy specimens. Five cases of hepatic EH diagnosed in needle biopsies encountered during the period from 1999 to 2010 in Beijing Cancer Hospital were retrospectively reviewed. The specimens were formalin-fixed, paraffin-embedded and stained with hematoxylin and eosin. Immunohistochemical study was also carried out. All the 5 patients were females. The age ranged from 23 to 47 years (mean = 39 years). The tumors in 4 patients were multiple and diagnosed as "metastasis" on ultrasound examination. The blood test results in all of the 5 patients were normal. Histologically, the tumor cells had an epithelioid appearance and were arranged in cords, solid nests or isolation, amongst a myxoid or hyaline matrix. The tumor cells contained scattered intracytoplasmic vacuoles which sometimes harbored red blood cells. There was no evidence of significant cellular pleomorphism, high mitotic activity and necrosis. Immunohistochemically, all of the 5 cases were positive for at least two endothelial markers (CD31, CD34 and factor VIII-related antigen). Smooth muscle actin was expressed in 1 case. The diagnosis of hepatic EH can be established in needle biopsy specimens. The histologic pattern, when coupled with immunohistochemical findings, is useful in arriving at the correct diagnosis.

Primary hepatic neuroendocrine carcinoma with nonbacterial thrombotic endocarditis: A case report

Nonbacterial thrombotic endocarditis (NBTE) is occasionally found in patients with carcinoma (lung or gastrointestinal) or debilitating diseases. Definite diagnosis of NBTE should be performed by autopsy. It is difficult to diagnose NBTE if patients have no clinical signs of thromboembolism. Here, we present a case of primary hepatic neuroendocrine carcinoma with NBTE as proven by autopsy.

Myxoid liposarcoma: A case report of a sentinel metastasis to the parotid gland with molecular confirmation

Myxoid liposarcoma is a subtype of liposarcoma with a predilection for the deep soft tissues of the extremities that accounts approximately for 10% of all adult soft tissue sarcomas. We report a case of a metastatic myxoid liposarcoma to the parotid gland, with fine-needle aspiration cytology correlation and molecular characterization. The lesion was diagnosed in a 53-year-old Hispanic male who presented with a left posterior thigh mass. A core needle biopsy established the diagnosis of myxoid liposarcoma. The patient underwent limb-sparing, wide local excision of the malignancy and later presented with an initial metastatic lesion to the parotid gland. The diagnosis of metastatic myxoid liposarcoma was rendered by fine-needle aspiration cytology with cell block preparation, and molecular confirmation. Although myxoid/round cell liposarcomas are classically described as having minimal pleomorphism on cytologic material, we encountered significant pleomorphism in our case. Therefore, a diagnosis of myxoid/round cell liposarcoma should still be a diagnostic consideration even if markedly pleomorphic cells are seen in fine-needle aspiration biopsies.

Mitochondrial alterations in children with chronic liver disease

Background Over recent years it has become apparent that the hepatocyte mitochondrion functions both as a cause and as a target of liver injury. Resultant dysfunction of mitochondria yields deficient oxidative phosphorylation, increased generation of reactive oxygen species, impairment of other metabolic pathways and activation of both necrotic and apoptotic pathways of cellular death. Methods This study was conducted on 26 children and adolescents with chronic liver disease who presented to or were following up in the Pediatric Hepatology Clinic, Children’s Hospital, Ain-Shams University. They were divided into three groups according to the aetiology of liver disease (GI = patients with Wilson’s disease (WD), GII = patients with chronic hepatitis C, GIII = patients with chronic liver disease other than Wilson’s and chronic hepatitis C). Ultrasound-guided gun liver biopsies were performed, under local anaesthesia for all the 26 patients, using a modified 18-gauge truecut needle. Two liver biopsy cores were taken from each patient. One for light and electron microscopic examinations and the other was immediately immersed in liquid nitrogen to be frozen and used for studying mitochondrial DNA deletions by PCR. Results Liver steatosis was higher in the group of patients with Wilson’s disease and other liver diseases. Electron microscopic examination of the mitochondria revealed significant mitochondrial pleomorphism in patients with Wilson’s disease and patients with chronic hepatitis C infection. Enlarged mitochondria were found to be more prevalent among patients with chronic hepatitis C infection. Three of our patients (11.53%) had mitochondrial DNA deletions. We developed a scoring system for mitochondrial affection in our patients, 7 patients (32%) were considered to have mild mitochondrial affection, 9 patients (41%) had moderate mitochondrial affection, while 6 patients (27%) had severe mitochondrial affection. Four of the studied patients had no mitochondrial affection. Conclusion Mitochondria affection is common in chronic liver disease. This mitochondrial affection might be responsible for some of the chronic liver disease manifestation such as easy fatiguability and steatosis.