Histopathological, immunohistochemical, and image analytic parameters characterizing the stromal component in primary and recurrent giant cell tumor of bone.

Giant cell tumor (GCT) of bone is a benign locally aggressive tumor whose biological behavior is unpredictable. Currently, there are no definitive clinical, histological, biochemical, or immunological parameters that can predict its behavior. This study was undertaken to examine whether delineation of reactive and neoplastic stromal component of GCT can help in this regard. 55 cases of GCT (30 primary, 25 recurrent) were subjected to histopathological grading, immunohistochemistry, and image analysis. Spindling of stroma was more frequent in recurrent GCT with 64% cases having more than 50% spindled stroma (p < 0.001). Number of mitosis/10 HPF and higher grade were more in recurrent GCT. Mean percentage positivity for CD68 (38.36%) and α1-ACT (70.86%) was higher in primary than recurrent GCT. PCNA and MiB-1 labeling indices were higher in recurrent (42.62% and 9.18%, respectively) than in primary group (24.75% and 7.7%, respectively). A single numerical parameter encompassing stromal cell population and its proliferation was derived as ratio of PCNA/CD68 and PCNA/α1-ACT. Both ratios were higher in recurrent (0.81 ± 0.38; 1.58 ± 1.50) than in primary GCT (0.58 ± 0.62; 0.34 ± 0.29) (p = 0.002; 0.01). On image analysis, parameters significantly different between the two groups were nuclear area and nuclear integrated optical density. It was thus concluded that recurrent GCT shows higher grade, increased mitosis, more spindling, fewer reactive components, and higher proliferation than primary GCT. Delineation of reactive component (α1-ACT positive) and proliferating component (PCNA positive cells) using immunohistochemistry with calculation of the PCNA/ACT ratio delivers more information than image analysis.

Laryngeal leiomyosarcoma masquerading as laryngeal carcinoma.

Laryngeal leiomyosarcoma is an exceedingly rare malignant tumour, with fewer than 50 reported cases in scientific literature. Diagnosis is based on immunohistochemistry, supplemented with ultrastructural studies, if required. It is aggressive and associated with variable survival outcomes. A 63-year-old man presented with hoarseness for 7 months and breathlessness for 3 months. Imaging showed a well-defined 3 cm glottic mass. Total laryngectomy was performed. The histopathological examination showed features of leiomyosarcoma. The index case has been presented owing to its rarity, variable clinical manifestations and diagnostic dilemmas and to stress upon the importance of ancillary techniques for confirmation.

Prediction of chemotherapy response by PET-CT in osteosarcoma: correlation with histologic necrosis.

BACKGROUND: The current standard for neoadjuvant chemotherapy (NACT) response evaluation in osteosarcoma is histopathologic necrosis (HN). However, it is accessible only after NACT completion and may get affected by confounding factors. Thus, noninvasive surrogate such as (18)Fluorine-Fluorodeoxyglucose-positron emission tomography-computerized tomography (PET-CT) scan would be useful to individualize therapy. METHODS: Thirty-one treatment naive osteosarcoma patients evaluated prospectively by PET-CT scan preceding and after 3 cycles of NACT and surgery during 2006 to 2008. Various anatomic and metabolic parameters of PET-CT scan were compared with HN (good response ≥90% HN) as reference standard. Receiver operating characteristic curves were generated to assess the best threshold and predictability. RESULTS: Median age was 17 years; 25 were male patients and 23 were nonmetastatic. Ten cases were good, whereas 21 cases were poor histologic responders. PET-CT parameters including post-NACT (2) and pre-NACT (1) standard uptake value (SUV)max ratio (SUV2:SUV1), SUV2, pre-NACT and post-NACT volumes (V1and V2), change in V after NACT, pre-NACT and post-NACT metabolic burden (MB) and change in MB after NACT correlated with HN. Two independent predictors were identified in stepwise multivariable analysis; if V1 ≤300 mL and SUV2:SUV1 ≤0.48, observed good histologic response proportions was 83%, whereas if V1 >300 mL and SUV2:SUV1 >0.48, it was 0%. CONCLUSIONS: NACT response can be predicted reliably by PET-CT scan early in disease course (even at baseline) and PET-CT parameters correlate well with HN. MB seems to be sensitive substitute for response evaluation. Independent predictors may have wider clinical applications if further validation can be done.

Primary mixed mullerian tumor of the vagina--a case report with review of the literature.

Malignant mixed mullerian tumor (MMMT) is a rare entity. The commonest site of this tumor in the female genital tract is the uterus followed by cervix. Primary MMMT of vagina is extremely rare. We are reporting this rare entity, with a brief review of the literature, in a 48-year-old perimenopausal female who presented with a history of passage of urine per vagina. On pelvic examination, a polypoidal mass arising from the anterior wall of the vagina was identified. Histopathological examination revealed the biphasic nature of the tumor. Immunohistochemistry confirmed the diagnosis of MMMT of vagina. To conclude, although rare, clinicians, oncologists, and pathologists should identify this malignant tumor for appropriate treatment and management.

Extraskeletal mesenchymal chondrosarcoma of the pleura: report of a rare case.

Mesenchymal chondrosarcoma is a rare aggressive variant of chondrosarcoma that frequently occurs in extraskeletal location. A 28-year-old female presented with a history of dyspnea and fever and succumbed to her illness before a conclusive diagnosis was established. An autopsy performed revealed the presence of an extraskeletal mesenchymal chondrosarcoma (ESMC) involving the pleura. Only one case of ESMC of the pleura has been reported previously. Herein, we report the second case of ESMC of the pleura.

Role of MRI in osteosarcoma for evaluation and prediction of chemotherapy response: correlation with histological necrosis.

BACKGROUND: Histological necrosis, the current standard for response evaluation in osteosarcoma, is attainable after neoadjuvant chemotherapy. OBJECTIVE: To establish the role of surrogate markers of response prediction and evaluation using MRI in the early phases of the disease. MATERIALS AND METHODS: Thirty-one treatment-naïve osteosarcoma patients received three cycles of neoadjuvant chemotherapy followed by surgery during 2006-2008. All patients underwent baseline and post-chemotherapy conventional, diffusion-weighted and dynamic contrast-enhanced MRI. Taking histological response (good response ≥90% necrosis) as the reference standard, various parameters of MRI were compared to it. A tumor was considered ellipsoidal; volume, average tumor plane and its relative value (average tumor plane relative/body surface area) was calculated using the standard formula for ellipse. Receiver operating characteristic curves were generated to assess best threshold and predictability. After deriving thresholds for each parameter in univariable analysis, multivariable analysis was carried out. RESULTS: Both pre-and post-chemotherapy absolute and relative-size parameters correlated well with necrosis. Apparent diffusion coefficient did not correlate with necrosis; however, on adjusting for volume, significant correlation was found. Thus, we could derive a new parameter: diffusion per unit volume. CONCLUSION: In osteosarcoma, chemotherapy response can be predicted and evaluated by conventional and diffusion-weighted MRI early in the disease course and it correlates well with necrosis. Further, newly derived parameter diffusion per unit volume appears to be a sensitive substitute for response evaluation in osteosarcoma.

Potter syndrome with an unusual cardiac anomaly.

Potter syndrome is a congenital anomaly characterised by bilateral renal agenesis, pulmonary hypoplasia, cardiac, skeletal abnormalities and maternal oligohydramnios. Here we report a case of Potter syndrome with bilateral renal agenesis, pulmonary hypoplasia and complete transposition of the great vessels, which had been identified during a post-mortem examination. Although cardiac anomalies are known to exist with Potter syndrome, complete transposition of the great vessels has not been reported in the literature.

Low grade central osteosarcoma--a diagnostic dilemma.

Low grade central osteosarcoma is a rare primary bone tumor. This fibro-osseous lesion shares some radiological and histopathological resemblance with fibrous dysplasia, which is a more common entity. Thus it may be mistaken as fibrous dysplasia and may receive inadequate treatment resulting in a more malignant recurrent bone tumor. We present a case of low grade central osteosarcoma of the right tibia, which was initially treated as fibrous dysplasia with curettage and bone grafting. Three years later the tumor recurred with greater soft tissue and bony involvement but without metastasis. The patient was treated with above knee amputation and has been asymptomatic for the last two years.

Acral and palmo-plantar hyperpigmentation in a patient with disseminated hepatocellular carcinoma.

Capecitabine (5-fluorouracil prodrug) is being evaluated for the management of hepatocellular carcinoma, and is associated with a peculiar skin reaction called hand and foot syndrome (HFS). We describe one patient with HCC and drug-induced HFS.

Primary colonic liposarcoma causing colo-colic intusussception: a case report and review of literature.

INTRODUCTION: Liposarcomas are most common soft tissue sarcomas usually seen in deep soft tissues of extremities and retroperitoneum. Although secondary involvement of gastrointestinal system can occur in cases of retroperitoneal tumours, liposarcomas primarily involving the gastrointestinal tract are extremely uncommon. Intusussception refers to telescoping of contiguous segments of intestine causing obstruction. Colo-colic intusussception is rare in adults and usually associated with a malignant etiology, usually epithelial malignancies (adenocarcinomas). DISCUSSION: Colonic liposarcomas leading to colo-colic intussusception are extremely rare, with only six cases reported in the literature. CONCLUSION: In this report, we describe this rare occurrence in a 66-year-old lady and highlight the diagnostic difficulties such cases can pose along with a review of relevant literature.

Invasive colonic mucormycosis in early induction therapy of childhood acute lymphoblastic leukemia.

Fungal infections occur in patients who are severely immunocompromised with profound and prolonged neutropenia. We report a patient of acute lymphoblastic leukemia who developed nonspecific abdominal symptoms within two days after the onset of neutropenia in the early induction phase of chemotherapy, which was later found to be due to intestinal mucormycosis and resulted in a fatal outcome.