Case report: Caecal epiploic appendagitis mimicking acute appendicitis.

INTRODUCTION: Acute appendicitis is a very common cause of abdominal pain which is optimally treated surgically. On the other hand, epiploic appendagitis is a self-resolving condition typically managed with analgesia alone, which can also present with severe abdominal pain. Both can present similarly and be difficult to distinguish. CASE: A 38 year old male presented with 2 days of periumbilical and right iliac fossa pain, with features of localised peritonism on physical exam. Inflammatory markers were only very mildly elevated but a computed tomography scan demonstrated findings in keeping with mild acute appendicitis. OUTCOME: Laparoscopic appendectomy demonstrated a torted epiploic appendage immediately adjacent to the vermiform appendix. The appendix had very mild inflammatory changes at the base adjacent to the appendage, but otherwise normal macroscopic appearance. Histopathology confirmed periappendicitis without features of acute appendicitis. CONCLUSION: Right sided epiploic appendagitis can mimic acute appendicitis, and in select patients with right iliac fossa pain there may be a role for serial observation to avoid an unnecessary operation.

The Pathophysiology and Impact of Inflammation in Nonscarred Renal Interstitium: The Banff i Lesion.

BACKGROUND: Interstitial inflammation (i-INT) is the driver of T-cell-mediated rejection. Its causes, pathophysiology, kinetics, and outcomes are poorly documented. METHODS: The role of i-INT was evaluated in 2055 biopsies from 775 renal transplant recipients. RESULTS: i-INT was present in 374 (18.2% prevalence) from acute and subclinical rejection (67.4%); interstitial fibrosis and tubular atrophy (14.4%); BK virus nephropathy (BKVAN) 9.9%; and acute tubular necrosis (ATN with i-INT) in 5.9% of cases. i-INT was predicted by prior T-cell-mediated rejection and BKVAN, human leukocyte antigen mismatch, cyclosporine therapy, and indication biopsy for dysfunction. It correlated with tubulitis, arteritis, and antibody markers within concurrent histology (P < 0.001). After treatment, renal functional recovery was best with histological ATN, milder i-INT, and early posttransplant biopsy times. The initial histological improvement of inflammation depended on baseline i-INT severity. Complete resolution to Banff i0 was predicted by early biopsy time, antilymphocyte therapy, recipient age, and medication compliance (all P < 0.001). Clearance i-INT was followed by delayed resolution of tubulitis (P < 0.001). i-INT was associated with histological ATN, renal dysfunction, and increased incident fibrosis on sequential pathology. Progressive fibrosis following related-rejection i-INT was dependent on tubulitis using multivariable analysis. In contrast, fibrogenesis after BKVAN or ATN was unrelated to inflammation. i-INT cases were followed by recurrent rejection in 35.3%, increased graft loss, and greater patient mortality. Multiple complementary outcome analyses determined the optimal lower diagnostic threshold for inflammation was Banff i1 score. CONCLUSIONS: i-INT is a heterogeneous pathological phenotype that results in adverse functional and structural outcomes, for which active and robust therapy should be considered.

A histologic evaluation of the laparoscopic adjustable gastric band capsule by tissue sampling during sleeve gastrectomy performed at different time points after band removal.

BACKGROUND: Laparoscopic sleeve gastrectomy (SG) is gaining popularity as a revision option after failed laparoscopic adjustable gastric banding (LAGB). Data have shown that single stage revisions may be associated with a higher complication rate. A histologic basis for this observation has not been studied. The objective of this study was to document the histologic properties of the LAGB capsule across the gastric staple line after SG at various time points after LAGB removal. METHODS: Gastric sleeve specimens of all LAGB to SG revisions were identified from January to May 2013 and underwent histologic evaluation of the LAGB capsule. Single blinded pathologist interpretation was performed, with inflammation, fibrosis, neovascularization, foreign body (FB) reaction, and wall thickness assessed semi-quantitatively and scored from 0-3. Based on combined features, an attempt was made to predict the timing of revision surgery. RESULTS: The study identified 19 revisions performed for inadequate excess weight loss or weight regain. The mean age for revision was 44 (19-65). The minimum time to revision was 42 days, the longest 1,188 days. There were no surgical complications. Varying degrees of inflammation and fibrosis were common features at all times. Angiogenesis, neovascularization and FB reaction were prominent in revisions performed before 80 days. The gastric wall was thicker during early revision. The optimal time to perform revision was difficult to determine. CONCLUSIONS: LAGB caused varying degrees of inflammatory and FB reaction that time did not fully resolve. The lower leak rates observed with delayed revisions do not appear to be attributable to gastric histology.

Lymphangiosarcoma arising after 33 years within a background of chronic filariasis: a case report with review of literature.

Cutaneous angiosarcoma or lymphangiosarcoma represents an uncommon aggressive tumor known to arise on a background of chronic lymphedema secondary to various etiologies, principally following surgery or irradiation. There have been rarely reported cases of angiosarcoma following infective conditions that eventuate with lymphatic stasis. We report a case of angiosarcoma arising after 33 years within a background of filariasis. Awareness of this association can lead to early diagnosis and appropriate treatment of this potentially fatal malignant tumor.

Parvovirus B19 presenting with persistent pancytopenia in a patient of T-ALL post induction chemotherapy diagnosed on bone marrow examination.

Manifestations of parvovirus B19 vary even in the normal host from asymptomatic or subclinical infection to a spectrum of illness with symptoms during viremic and immune complex mediated stage of disease. We report the morphological findings of parvovirus B19 infection (confirmed on serology) in a patient of T-acute lymphoblastic lymphoma (T-ALL) who underwent induction phase of chemotherapy (MCP 842 protocol). Persistent pancytopenia in the bone marrow aspirate with mild increase in blasts was thought to be due to failure to achieve marrow remission. However, giant pronormoblasts with prominent intranuclear inclusions confirmed on trephine biopsy led to the suspicion of parvovirus B19 infection which was later confirmed on serology. This case is presented to report the rarely seen classical morphological feature of parvovirus infection on bone marrow examination which was incidentally the first investigation to diagnose the viremic phase of the infection, indicating that a high index of suspicion needs to be kept in mind while examining bone marrows of susceptible patients.

A study of pathologic risk factors in postchemoreduced, enucleated specimens of advanced retinoblastomas in a developing country.

CONTEXT: Advanced cases of retinoblastoma are treated with chemoreduction followed by enucleation. Further adjuvant therapy is recommended in patients with known pathologic risk factors (PRFs). OBJECTIVES: To determine the PRFs in enucleated specimens after chemoreduction and their association for adverse events of recurrence, metastasis, or death. DESIGN: This was a retrospective study of 77 enucleation specimens from patients treated between January 2000 and September 2008 with prior chemoreduction that were accessioned in the pathology department of a tertiary referral cancer center with an average follow-up of 24 months. Various PRFs were noted and their association with the development of an adverse event was recorded. RESULTS: Of 77 patients, (male to female ratio, 51∶26), the incidence of overall PRF was 51.9%, and retrolaminar optic nerve invasion (32.5%), optic nerve cut margin (12.9%), massive choroidal invasion (26%), scleral invasion (23.4%), vitreous seedings (44.2%), and anterior segment invasion (20.8%). Undifferentiated tumor (>60%) was seen in 60.3% of cases (41 of 68 patients with differentiation available). Adverse event occurred in 18 of 72 patients with available follow-up (25%). Retrolaminar optic nerve invasion, optic nerve cut margin involvement, and scleral invasion were independent prognostic factors predicting the occurrence of an adverse event. Undifferentiated tumor (>60%) was a significant risk factor in univariate analysis, which is the unique feature in this study. CONCLUSIONS: Classic PRF with the addition of a predominant presence from the undifferentiated component were associated with adverse outcomes in retinoblastoma treated with anterior chemotherapy. The latter may represent chemoresistant clones and more intensive adjuvant chemotherapy may be warranted in these patients.

Sarcomatoid (spindle cell) carcinoma of the head and neck mucosal region: a clinicopathologic review of 103 cases from a tertiary referral cancer centre.

Sarcomatoid carcinomas are biphasic tumors proven to be monoclonal dedifferentiated forms of conventional squamous carcinomas. This study evaluates their clinicopathologic characteristics in head and neck mucosal sites and the problems in distinguishing them from other spindle cell tumors. A total of 103 cases with a confirmed diagnosis of sarcomatoid carcinoma accessioned in the pathology department of a tertiary referral cancer centre over a period of 7 years (2004-2010) were studied. An algorithm used for their diagnosis is presented. Ages of the patients were 22-90 years (median 53 years), and male:female ratio was 3.7:1. Site distribution was oral cavity (n = 65, 63.1%), larynx (18, 17.5%), oropharynx/hypopharynx (12, 10.7%), maxilla (6, 5.8%) and metastatic nodes (2, 1.9%). A large number of patients (95%) presented with a mass lesion of less than 1 year duration. Histopathologically, epithelial differentiation was evident on morphology in 48 (46.6%) cases, only on IHC in 34 (33%) cases, and in 21 (20.4%) no epithelial differentiation was seen. Typically, tumors were polypoidal (92, 89.3%) and ulcerated (95, 92.2%) with cells arranged predominantly in fascicles (59, 57.3%) or storiform pattern (17, 16.5%) amidst collagenous (50, 48.5%) or myxoid matrix (35, 34%). Anaplasia (2+/3+) and mitosis >10 per 10 HPF were noted in 96 (93.2%) cases. IHC was done in 82 cases; 55 (66.7%) showed positivity for epithelial markers with aberrant expression of mesenchymal markers in 43 (41.7%). Diagnosis of sarcomatoid squamous carcinoma is challenging because of overlapping histopathological features with other spindle cell tumors. Understanding their clinicopathologic characteristics facilitates their diagnosis and appropriate clinical management.

ALK+ anaplastic large cell lymphoma with cohesive, perivascular arrangements on cytology, mimicking a soft tissue sarcoma: a report of 2 cases.

BACKGROUND: An accurate recognition of a lymphoma at an extranodal site is essential to avoid unnecessary excisions. Fine needle aspiration cytology (FNAC) has been recognized as a useful tool in the primary diagnosis of soft tissue tumors. An anaplastic large cell lymphoma (ALCL), occurring in soft tissues, poses a diagnostic challenge. We present the cytomorphology of 2 cases of anaplastic lymphoma kinase (ALK)+ ALCL that displayed a perivascular arrangement, thereby mimicking a sarcoma. CASES: The patients were aged 16 and 9 years. While the former presented with multiple nodular soft tissue and bony lesions over the body, the latter complained of an axillary lump. FNAC smears in both cases showed pleomorphic cells, arranged in loosely cohesive groups around blood vessels and scattered singly, with prominent nucleoli and abundant, finely vacuolated cytoplasm. Interspersed were a few cells with embryoid nuclei. Differential diagnoses included a rhabdomyosarcoma and a lymphoma. On biopsy and immunohistochemistry, tumor cells were positive for vimentin, LCA, EMA, CD30 and ALK. CONCLUSION: ALCL should be considered in the differential diagnosis of pediatric soft tissue tumors, especially in cases with multifocal involvement. The presence of cohesive, perivascular arrangements on FNAC should not detract a cytologist for keeping this possibility in mind. Further, a careful search for the typical "hallmark" cells should be made.

Evaluation of pediatric abdominal masses by fine-needle aspiration cytology: a clinicoradiologic approach.

The pathologist forms a very important part of the clinical team in the management of pediatric intra-abdominal masses in giving a rapid, accurate diagnosis for these potentially curable tumors. Fine-needle aspiration cytology (FNAC) is an invaluable tool in this regard when interpreted with clinicoradiologic parameters. With this in mind, we decided to evaluate the role of FNAC in pediatric abdominal masses in our institution. A total of 83 of 105 FNAC accessioned in the pathology department over 5 years (2003-2007) were studied. These included only cases where a diagnosis could be offered on cytology. Detailed clinicoradiological features were obtained from hospital records. Cytomorphological features examined included cellularity, architectural pattern, background, key cellular details. Immunocytochemistry were done where necessary. Lesions diagnosed on FNAC included Wilms' tumor (19), lymphoma (10), neuroblastoma (6), hepatoblastoma (5), PNET (5), rhabdomyosarcoma (2), DSRCT (2), germ cell tumor (6), and miscellaneous tumors (7). Definite diagnosis could be offered on cytomorphology in 74.7% (62) cases, while in 25.3% (21) cases only a diagnosis of round cell tumor could be offered. Concordance with final histopathology and biochemical parameters was subsequently obtained in 79/83 (95.5%) of cases. A clinically relevant classification is possible on FNAC in pediatric abdominal tumors when interpreted with clinicoradiologic parameters. This obviates the need for a more time-consuming biopsy procedure in critical situations and in stage II nephroblastoma where it is contraindicated.

Metastatic giant cell tumor of bone: are there associated factors and best treatment modalities?

UNLABELLED: Giant cell tumors of bone are sometimes locally aggressive and may metastasize, although uncommonly. We attempted to identify associations of clinical and histopathologic parameters with metastasis, the long-term outcome with metastases, and the best treatment. We identified distant metastases in 24 of 470 patients with giant cell tumors during a 20-year period. The median age of these 24 patients at presentation was 26 years (range, 16-76 years), and the male:female ratio was 1.6:1, with no predilection for primary site. Metastasis occurred at a mean of 2 years (range, 4 months-11 years) after initial diagnosis. Sites for distant metastases were the lung (21 of 24 patients), scalp, calf muscle, and regional lymph nodes. The 24 patients had a mean followup of 3.5 years (range, 0-16 years). Thirteen of the 24 patients has local recurrence before or at the time of metastasis. Two patients refused treatment, eight underwent metastasectomy, and 14 were inoperable (four had chemotherapy, 10 were treated symptomatically). We observed disease progression with hemoptysis in one of 14 patients. None of the patients died of their metastatic disease. None of the risk factors we studied was associated with metastasis in giant cell tumors. Although the overall outcome was favorable, metastasectomy is recommended where feasible. LEVEL OF EVIDENCE: Level IV, therapeutic study. See Guidelines for Authors for a complete description of levels of evidence.

Extraconal orbital tumors in children--a spectrum.

Orbital masses in children are uncommon but extremely challenging problems for clinicians and pathologists due to their critical location and availability of limited diagnostic material. We analyzed 47 specimens comprising biopsies, excision specimens, and FNAC of extraconal pediatric orbital masses (excluding retinoblastoma) accessioned in the pathology department over 5 years in a tertiary referral cancer center. Immunohistochemistry (IHC-74%) and molecular methods (one case) were done where necessary. The chief presenting symptom was proptosis in 55.3% patients and radiologically 53.8% malignant tumors showed extraorbital extension. A diagnostic algorithm was formulated to assess which cases needed pathology evaluation. Malignant round cell tumors (76.6%), chiefly embryonal rhabdomyosarcoma (51%), benign spindle cell neoplasms, and infectious lesions (tuberculosis, fungal infections), were seen. Of the malignant tumors, those confined to the orbit achieved good treatment response and had an event-free follow-up while those with extraorbital spread had poor outcome. Pediatric orbital masses range from completely treatable infectious lesions, surgically resectable benign neoplasms to aggressive malignancies requiring chemotherapy and radiotherapy. Pathologists play a key role in distinguishing these on small biopsy material and expediating accurate treatment thus saving the vision or life of a patient.

Diagnostic dilemmas in fine needle aspiration cytology of an ectopic kidney: a case report.

BACKGROUND: Congenital anomalies are easily diagnosed on radiology and rarely have an unusual presentation requiring an invasive diagnostic procedure. Fine needle aspiration cytology (FNAC), though a well-established diagnostic technique for abdominal and retroperitoneal lesions, is fraught with several challenges and pitfalls. CASE: We report a case of a 39-year-old woman with an ectopic kidney presenting as an abdominal mass. On FNAC the cellular aspirate was misinterpreted as a paraganglioma or metastatic carcinoma. CONCLUSION: This case highlights the fact that on FNAC, normal cellular components, especially from the kidney, provide a significant pitfall for overdiagnosis, potentially resulting in unnecessary surgical explorations.

Squamous differentiation in a sarcomatoid chromophobe renal cell carcinoma: an unusual case report with review of the literature.

We describe an extremely rare occurrence of a squamous differentiation in a sarcomatoid chromophobe renal cell carcinoma in a 45-year-old woman with nodal and lung metastasis at presentation. The tumor on histology showed all 3 components intimately admixed with each other, which to the best of our knowledge is the first such case to be reported in the literature. The renal pelvis was smooth walled and uninvolved. Kidney-specific cadherin was positive in the chromophobe renal cell carcinoma areas and negative in the sarcomatoid and squamous areas.

Primary PNET of maxilla: an unusual presentation.

Primitive neuroectodermal tumors (PNETs) are a type of small round cell tumors developing from migrating embryonal cells of the neural crest. Peripheral primitive neuroectodermal tumors (pPNETs) are less common with varying incidence of occurrence in head and neck region. Only 8 reported cases of primary PNET of maxilla are available in English literature. We report a case of 8-year-old boy diagnosed as pPNET of maxilla after detailed radiologic, histopathologic, including immuno-histochemical examination and molecular diagnosis using reverse transcription-polymerase chain reaction showing EWS-FLI1 translocation. The boy was treated with multiagent combination chemotherapy to be followed by definitive radiation therapy. A brief literature review of diagnosis and management of the previous 8 reported cases is done. In view of no definitive guideline for management of such cases, treatment on the lines of other pPNET is suggested.

Orbital medulloepitheliomas -- with extensive local invasion and metastasis: a series of three cases with review of literature.

Intraocular medulloepithelioma is a rare embryonal neoplasm which usually presents in childhood as a ciliary body mass. This tumor is usually confined to the orbit and rarely tends to be locally aggressive and metastatic. We describe three rare cases of metastatic malignant teratoid medulloepithelioma. The patients were males aged 7, 16 and 25 years with right eye involvement in all the cases. All patients had local recurrence (multiple in two) and metastasis to the intraparotid and cervical lymph nodes. Distinction from round cell tumors and primary salivary gland neoplasms occurring in the parotid gland is strongly warranted, especially in biopsy samples, because the biological behaviour and treatment of medulloepithelioma differs greatly from the above lesions.

Rare presentation of Kyrle's disease in siblings.

BACKGROUND: Kyrle's disease is a rare variant of primary perforating dermatosis. Its occurrence in a familial setting, especially in children, is extremely uncommon. Similar appearing skin lesions have been described in adults, secondary to metabolic disorders, infective agents as well as exposure to chemicals. We present a rare case of this genodermatosis in two siblings. MATERIALS AND METHODS: Two siblings of a non-consanguineous marriage came with generalized discrete papular lesions with a central keratotic plug. All biochemical and serological investigations were within normal limits. Serial sections of the biopsy revealed typical epidermal invaginations filled with parakeratotic debris and perforation into the dermis with accompanying granulomatous reaction. RESULTS AND CONCLUSIONS: A careful history, detailed routine investigations and serial sections of the skin biopsy are required to demonstrate the typical morphology and stages of evolution of Kyrle's disease. This helps to differentiate the rare primary Kyrle's disease from other primary and secondary keratotic lesions. Due to the familial occurrence, screening of relatives of an index case is recommended.

Clear cell sarcoma of the kidney--a study of seven cases over a period of three years.

UNLABELLED: Clear cell sarcoma of the kidney (CCSK) can display diverse morphological patterns and mimic various other pediatric renal tumors. An accurate diagnosis of this tumour is important considering the therapeutic and prognostic implications. AIM: The present study was undertaken to describe the various histological patterns of CCSK. The histology of 7 cases and the available case files of CCSK accrued over a period of3 years were reviewed. Immunohistochemical (IHC) stains were performed in 3 cases. The histological patterns observed in this study were classical (observed in 4 cases), epithelioid trabecular, myxoid, palisading and hyaline sclerosis types. IHC revealed reactivity to vimentin and non-reactivity to cytokeratin, desmin, smooth muscle actin, neuron specific enolase (NSE) and S-100 protein. Since CCSK is essentially a histological diagnosis, the importance of an accurate diagnosis of CCSK by a pathologist cannot be overemphasized. This study describes the various histological patterns that can be observed in CCSK.

Metastatic adenocarcinoma presenting as an inguinal hernia: a case report and review of literature.

A 61 year old man presented with an inguinal hernia with no other significant symptoms. Histopathological examination of the hernial sac revealed metastatic deposits of a mucin secreting adenocarcinoma which was confirmed by subsequent tumor marker levels. Patient was put on chemotherapy for disseminated adenocarcinoma and is tolerating it well. This case emphasizes the need to carefully examine all hernial sacs received for pathological examination.