Cyclin-D1 utility in classification of endometrial stromal sarcomas and segregation from other uterine sarcomas

Context: Endometrial stromal sarcomas [ESSs] are rare mesenchymal tumors of uterus which accounts for less than 10% of all uterine sarcomas. Gene rearrangement studies unveil their genetic heterogeneity with the discovery of specific YWHAE-FAM22 gene fusion. According to recent WHO classification of endometrial stromal tumors all Endometrial stromal sarcomas with this fusion are labeled as high grade-Endometrial Stromal Sarcomas [HG-ESS]. Immunohistochemically HG-ESSs with YWHAE- FAM22 translocation express nuclear Cyclin D1 positivity which can be used as a surrogate immunohistochemical marker for this translocation

Objective: Determine diagnostic utility of Cyclin D1 as a marker to segregate high grade- Endometrial Stromal Sarcomas [HG-ESS] from low grade-Endometrial Stromal Sarcomas [LG-ESS] and undifferentiated uterine sarcoma [UUS] and from other uterine sarcomas including leiomyosarcomas and carcinosrcomas

Materials and Methods: Cases of Endometrial stromal sarcomas, leiomyosarcomas and carcinosarcomas received from Jan 2010- Jan 2015 were reviewed and immunostained with Cyclin D1, CD-10, ER, PR and Desmin. Nuclear positive or negative staining for Cyclin D1 was noted

Results: Total 36 cases of uterine sarcomas including 10 cases of ESS, 20 cases of leiomyosrcoma and 6 cases of carcinosarcoma were retrieved from surgical database. Out of ten cases of endometrial stromal sarcomas, five were low grade, two were high grade and three were undifferentiated sarcomas. Cyclin D1 was expressed in three cases previously diagnosed as low grade, high grade and undifferentiated sarcomas respectively. Cyclin D1 positive undifferentiated uterine sarcoma [UUS] also revealed diffuse CD-10 positivity. Other two cases with a positive Cyclin D1 expression revealed a patchy CD-10 positivity. Negative expression of Cyclin D1 was noted in all cases of leiomyosrcoma and carcinosarcoma

Conclusions: Endometrial stromal sarcomas are relatively uncommon tumors and can pose diagnostic problems for pathologists. Smooth muscle tumors [leiomysrcomas] and carcinosarcomas are among the top differentials. Final diagnosis usually requires immunohistochemistochemical analysis with a panel of immunomarkers. Cyclin D1 expression along with patchy CD-10 positivity favors HG-ESS over LG-ESS and UUS. Cyclin D1 positivity along with diffuse CD-10 expression favors Undifferentiated Uterine Sarcoma. Negative expression in other uterine sarcomas makes it a specific marker for ESSs. It is suggested that Cyclin D1 along with CD-10, ER, PR and Desmin should be added in immunohistochemical panel of high grade uterine sarcomas especially if patchy expression of CD-10 is noted. It is to make a provisional diagnosis of YWHAE-FAM22 associated HG-ESS when fluorescence in situ hybridization [FISH] and reverse transcriptase polymerase chain reaction [RT-PCR] are not available to make a final diagnosis

Adenoid cystic carcinoma of the trachea

DAdenoid cystic carcinoma is a malignant tumor of salivary glands. It is extremely rare in trachea worldwide. They arise in the subepithelioal minor salivary glands, grow slowly and metastasize by perineural invasion. Patient usually presents with dyspnea. It is diagnosed by histopathological evaluation of biopsy taken by endoscopy. Its treatment includes surgical resection with or without radiotherapy. They frequently recur after initial treatment

Synchronous Development of Gastrointestinal stromal tumor and arteriovenous malformation in the Jejunum

This case report is about synchronous development of gastrointestinal stromal tumors [GISTs] with AV malformations [AVMs]. A 23-year-old pale woman presented with melana and anemia. Abdominal computed tomography Non Enhanced Contrast [CT-NEC] revealed a gut wall thickness in left hypochondrium and multiple focal calcifications. Contrast enhanced computed tomography [CECT] revealed non-homogenous enhancement with circumferential gut wall thickening and narrowing lumen plus AV malformations in the gut wall giving impression of mass in the jejunum with AV malformation. An exploratory laprotomy was performed. The resected jejunum showed a protruding solid mass. The mass was confirmed to be a spindle cell type GIST and was intermingled with the AVM. To our knowledge, this is the second case of an AVM associated with a GIST. First case was reported in 2008 by Shim SH et al [1]

spectrum of inflammatory and developmental odontogenic cysts

To study the spectrum of inflammatory and developmental odontogenic cysts. Epithelial lined cysts of the oral cavity are among the most commonly encountered oral diseases from both the clinicians and pathologists perspective. To reach a specific diagnosis a multi team approach is necessary which includes radiologists, surgeons and pathologists. Two major categories of cysts exist; odontogenic and non-odontogenic. The most commonly encountered odontogenic cysts are radicular or periapical cyst; which may occur in any age group but common in 3[rd] and 4[th] decade. The second common type is dentigerous cyst that accounts for 17% of all the odontogenic cysts. Keratocysts account for 10% of all odontogenic cyst and can have more chances of recurrence. 60 cases sent to the Pathology Department during Sept. 2006 to Dec 2009 were included in the study, after observing inclusion and exclusion criteria. After gross examination the sections were routinely processed and slides stained with Hand E. All the slides were thoroughly examined by two consultant histopathologists and postgraduate pathology resident. 50% were radicular cysts, 36% were dentigerous cysts, keratocysts and eruption cysts accounted for 8% and 5% respectively. In one case of dentigerous cyst ameloblastoma was diagnosed and one case was diagnosed at maxillary antrum, an unusual site for dentigerous cysts

Hair dye poisoning, a cause of causing acute renal failure

We report a 22 year patient who developed acute renal failure following the ingestion of a hair-dye with a suicidal intent. He was managed by hemodialysis and other symptomatic measures. His renal biopsy showed acute interstitial nephritis along with granulomas and giant cells. Hair dyes contain a host of chemicals that can cause rhabdomyolysis, laryngeal edema, severe metabolic acidosis and acute renal failure. Intervention at the right time has been shown to improve the outcome

Touch impression cytology versus frozen section as intraoperative consultation diagnosis

The efficacy of Touch Imprint Cytology in the diagnosis of various pathological processes was evaluated and compared with Frozen Section in intraoperative consultation. We examined 60 cases from various sites of the body that were referred for intraoperative consultation. All cases were analyzed by Touch Imprint Cytology followed by frozen section. We found diagnostic accuracy of both procedures comparable. The diagnostic accuracy in distinguishing benign from malignant lesions by combined procedures was 100%. There were no false positive or false negative cases. For diagnosing specific subtypes of malignancy, the diagnostic accuracy of each method alone was 96.6% with a sensitivity of 86% and specificity of 100% and the combined sensitivity 90%. Touch Imprint Cytology provided better cellular morphology and fewer artifacts. On the other hand Frozen Sections provided more tissue architectural details but frequently hampered by freezing artifacts. We conclude that both Touch Imprint Cytology and Frozen Sections are dependable intraoperative consultation diagnostic modalities. Touch Imprint Cytology alone may provide a correct diagnosis in vast majority of cases with minimal expense and without the need of sophisticated cryostat machine thus making it quite suitable for many hospitals where cryostat machines are not available

Prostatic phabdomyosarcoma in an infant with neurofibromatosis

we are reporting an unusual case of a tumor in early infancy causing complete urinary obstruction. The patient was an infant with external signs of neurofibromatosis who presented with acute urinary retention prostatic rhabdomyosarcoma and was managed by total cystectomy and urinary diversion

Twin fetus in fetu-a very rare entity: a case report with review of literature

1] To report a very rare condition of conjoined twining in fetus in fetu in an infant. From literature it appears to be the 4th case being reported. 2] To emphasize the importance of proper examination of the fetus in fetu grossly by doing extensive dissection, radiologically and detailed histological tissue examination from several sites. Design: Detailed report of an unusual intra-abdominal mass in an infant. Setting: Department of Pediatric Surgery, Children Hospital, PIMS, Islamabad. Successfully treated with surgical removal and interesting diagnosis emerged. Twin fetus in fetu [FIF] is a very rare anomaly due to an abnormal embryogenesis of fetus during pregnancy. Malformed fetus grows in the body of co-twin and it is frequently detected as an abdominal mass. There is no clear distinction between the term of FIF and fetiform teratoma [FT]. We are reporting a case in which a girl was referred as a case of nephroblastoma but at operation and on detailed examination of the specimen it turned out to be a case of twin fetus in fetu