Placental mesenchymal disease masquerading as molar pregnancy with a favourable maternal and fetal outcome.

Placental mesenchymal dysplasia (PMD) is an exceptionally rare placental anomaly characterised by placentomegaly and grape-like vesicles resembling partial mole on ultrasonography, yet it can coexist with a viable fetus. We present the case of a primigravida who presented at 22 weeks gestation with a suspected partial mole but with a normally growing fetus. The differential diagnoses considered included placental mesenchymal disease, partial mole and twin pregnancy with molar pregnancy. With normal beta HCG levels and prenatal invasive testing reports, a probable diagnosis of PMD was made, and after thorough counselling, the decision was made to continue the pregnancy. The pregnancy progressed until 37 weeks, culminating in the uneventful delivery of a 2.4 kg healthy male infant. Histopathology confirmed PMD. Early recognition and management of PMD pose significant challenges, given its rarity. Prenatal identification of PMD during both early and late gestation could avert unnecessary termination of pregnancy.

Cytohistological and Immunohistochemical Correlation of Cutaneous Mixed Tumors: A Series of Four Cases and Review of Recent Molecular Updates.

Fine needle aspiration cytology (FNAC) is an established diagnostic modality today, but its utilization in skin tumors is limited by the fact that most of these lesions are small and easily accessible for clinicians to perform an excision biopsy. As a result, our knowledge of the cytological features of some of the uncommonly encountered skin tumors is still lacking. The aim of this study was to correlate the cytological features of cutaneous mixed tumors (CMTs) with histological and immunohistochemical findings in four cases of CMT in commonly and uncommonly encountered locations. We also review the recent updates highlighting the various genetic rearrangements and newer more specific immunohistochemical markers described so far. This was a retrospective study, and all the slides were taken from our departmental archives. Case 1 was a 25-year-old male who presented with a gradually increasing painless swelling over the right angle of the mouth of 1.5 years duration. Case 2 was a 45-year-old male with swelling on the right forearm for the last three years. Case 3 was a 35-year-old female with a forehead swelling of one year duration. Case 4 was a 55-year-old female with left cheek swelling for two years. On clinical examination, all four nodular swellings were predominantly in the skin/subcutaneous tissue. On cytology, all cases showed abundant chondromyxoid material with clusters of benign epithelial cells and a fair number of predominantly singly scattered myoepithelial cells. The diagnosis of all four cases was further confirmed on histopathology and immunohistochemistry, and the findings correlated well with cytology. The cytological features of CMT closely correlate with their histopathological and immunohistochemical features. Newer immunohistochemistry (IHC) marker pleomorphic adenoma gene 1 (PLAG1) may be helpful in making a definitive diagnosis of CMT on cytology and cell block preparation along with a good clinical correlation. Complete surgical excision is the treatment of choice, and recurrence is rare.

Primary leiomyosarcoma of the vulva - a rare occurrence.

Vulval tumours are rare, representing merely 4% of all gynaecological neoplasms. 98% of vulvar lesions are benign, and only 2% are malignant. Of all vulvar malignancies, while squamous cell carcinoma is the most common malignancy, leiomyosarcomas of the vulva are extremely rare. Usually these tumours have nonspecific clinical manifestations, often leading to misdiagnoses of Bartholin cysts or abscesses. We describe a case of a 47-year-old woman who presented with a nonspecific painless swelling in the left vulva for 2 months which was diagnosed as leiomyosarcoma of the vulva on biopsy as well as resection.

Extramedullary Relapse of B Cell Acute Lymphoblastic Leukemia in Bilateral Breasts Treated with Whole Breast Radiotherapy.

Isolated extramedullary relapse of acute lymphoblastic leukemia (ALL) in the breast is extremely rare. We herein report a case of a 38-year-old female with B cell ALL, who had isolated extramedullary relapse initially in the left breast and subsequently in the right breast, 3 and 4 years, respectively, after hematopoietic allogenic stem cell transplantation. She was successfully salvaged with bilateral whole breast radiotherapy, 24 Gy/12 fractions/2.5 weeks. This brief report highlights the importance of awareness of extramedullary leukemic relapse in the breast as one of the differential diagnoses of breast masses in the context of ALL. Since these tumors are extremely radioresponsive, radiation therapy is a safe and effective treatment option for isolated extramedullary relapse of ALL in the breast.

Leptomeningeal carcinomatosis in a patient with recurrent unresectable squamous cell carcinoma of the retromolar trigone-a brief report.

BACKGROUND: The reported incidence of leptomeningeal carcinomatosis is 3-8% in patients with solid tumours. More commonly, it has been described in the setting of advanced cancers of the lung, breast and malignant melanoma. CASE PRESENTATION: A 50-year-old diabetic patient with recurrent unresectable squamous cell carcinoma (SCC) of the right retromolar trigone (rT4bN0M0) presented with severe low backache and weakness in bilateral lower limbs 20 days after the completion of concurrent chemoradiotherapy. Contrast-enhanced MRI of the spine showed multiple nodular enhancing leptomeningeal lesions at the lumbar level and an intramedullary T2/FLAIR-hyperintense longitudinal lesion involving the central cord from C2 to C7 vertebral levels, suggestive of leptomeningeal metastases. Cerebrospinal fluid (CSF) analysis revealed pleocytosis, elevated protein and markedly decreased glucose. The CSF cytology revealed scattered large atypical cells, suspicious for metastasis. Non-contrast MRI of the brain showed a T2/FLAIR-hyperintense lesion involving the right caudate nucleus suggestive of either an acute infarct with haemorrhagic transformation or a haemorrhagic brain metastasis. During assessment, he had high-grade fever and was started on empirical intravenous antibiotics (ceftriaxone, vancomycin and subsequently meropenem) in line with the management for acute bacterial meningitis. Gram staining of CSF did not demonstrate the presence of any bacteria and the specimen was sterile on culture. He did not respond to empirical antibiotics, had a progressive downhill course and eventually died due to aspiration pneumonia. CONCLUSION: This brief report highlights the importance of awareness of leptomeningeal carcinomatosis as a possible cause of backache with sensorimotor deficit and autonomic dysfunction in a previously treated case of head and neck SCC.

Primary Extra Mammary Paget's Disease of Vulva, With Apocrine Adenocarcinoma, Signet Ring Cell Differentiation and Distant Metastasis.

Objective: Extramammary Paget's disease (EMPD) with invasive carcinoma and distant metastasis is extremely rare. In vulva EMPD associated apocrine carcinoma with signet ring cell differentiation has not been described in the literature so far. Its slow evolution, varied clinical presentation and histological appearances, lead to difficulty in diagnosis of this disease. Case report: We hereby report a case of primary EMPD with invasive carcinoma and distant metastasis in a 59-year-old female who presented with erythematous indurated plaque over vulva. Histopathology revealed Paget cell infiltration throughout the epidermis with invasive carcinoma in dermis and liver metastasis on CECT. The immunohistochemical expressions of CK7, CK20, GCDFP-15, CEA, p40, CDX 2, Her-2/ neu, AR, ER, were examined to explicate the cellular differentiation of this carcinoma. According to the histological assessment, this case was diagnosed as primary EMPD with apocrine adenocarcinoma, signet ring cell differentiation, vulva. Conclusion: Owing to poor prognosis, a high index of clinical suspicion along with histological and immunohistochemical assessment is of utmost importance in arriving at final diagnosis.

Primary Splenic Pregnancy- A Rare but Imperative Cause of Hemoperitoneum - Case Report and Review of Literature.

Primary splenic pregnancy is an extremely rare form of extratubal ectopic pregnancy. These cases often cause splenic rupture in very early course of their gestation thereby presenting with hemoperitoneum in emergencies. Owing to the higher risk of exsanguination and death caused by hemoperitoneum, it is essential to diagnose these cases for proper management and better prognosis of the patients. We present the case of a 23-year-old female, gravida 2, para 1, live issue 1 presenting to the emergency outpatient department with acute abdomen and hemoperitoneum. There was no history of trauma. The patient had a positive urine pregnancy test and raised beta HCG levels. Emergency laparotomy revealed an otherwise unremarkable fallopian tube and ovary with a hemoperitoneum of 2.5 liters. A tiny splenic laceration was considered to be the source of bleeding and splenectomy was performed. Microscopy was suggestive of a primary ectopic pregnancy, spleen. Since hemoperitoneum in pregnancy is a rare but potentially fatal condition with a high risk of mortality, an accurate preoperative diagnosis is crucial in the management of such patients. The possibility of a ruptured extratubal ectopic pregnancy must be considered as one of the differential diagnoses of acute abdomen with hemoperitoneum in women of childbearing age.

Malignant transformation in mature cystic teratoma of the ovary: a retrospective study of eight cases and review of literature.

INTRODUCTION: Mature cystic teratoma (MCT) is the most common type of ovarian germ cell neoplasm, but occasionally it may undergo malignant change in any one of its elements. In this study, these rarely encountered tumors, occurring over a period of 25 years, were studied. MATERIAL AND METHODS: A retrospective, tertiary hospital-based study was carried out in all histopathologically diagnosed cases of MCT (230) of the ovary from January 1990 to December 2014. The clinicopathological features of malignant transformation (MT) in MCT of the ovary were retrieved from the archives of the Department of Pathology and were analyzed. RESULTS: Two hundred thirty (230) mature cystic teratomas of the ovary were found. MT was noted in eight of these cases, i.e. 3.5% of all the MCT. The mean age of the patients with MCT was 32.5 ±13.11 while the mean age of the patients with malignant transformation in MCT was 44.2 ±8.94 years. Grossly the mean size of the malignant teratoma was 11.7 ±2.7 cm, whereas it was 7.6 ±2.1 cm for mature cystic teratoma. Squamous cell carcinoma (SCC) was the most frequent MT seen in four out of eight cases, while one case showed an adenocarcinoma and the other a malignant melanoma, and two cases had transitional cell carcinoma. CONCLUSIONS: The rate of malignant transformation in MCT increases with age and is much higher in the postmenopausal age group. Moreover, although SCC is still the commonest, transitional cell carcinoma (TCC) may also develop not infrequently as malignancy apart from other rare differentiations such as adenocarcinoma or malignant melanoma in an MCT.

Clinicopathological Evaluation of 223 Cases of Mature Cystic Teratoma, Ovary: 25-Year Experience in a Single Tertiary Care Centre in India.

INTRODUCTION: Mature Cystic Teratoma (MCT) is the most common benign neoplasm of the ovary constituting 10-20% of all the ovarian tumours. Although, much has been published about ovarian teratomas, in English literature, there are very few retrospective cross sectional studies from India to understand the epidemiology of the disease. AIM: To evaluate the prevalence, clinicopathological characteristics and complications of MCT of the ovary. MATERIALS AND METHODS: This was a retrospective study of 223 cases of MCT of ovary received over a period of 25 years from 1990 to 2014. The data regarding age, size, laterality, gross, morphological features, complications and surgery performed was retrieved from the archives of pathology department. Descriptive statistics was performed using SPSS software version 22 and the results were expressed as percentages. RESULTS: MCT constituted 20.2% of the total ovarian neoplasms and 78.5% of all the germ cell tumours. The mean age of the patients with MCT was 32.5±13.11 years and the mean size of tumour was 8.6±3.1 cm. Torsion was the most common complication of these tumours. Malignant transformation of this tumour though rare was seen in 3.5% of the cases. CONCLUSION: MCT are most common tumours of the ovary. Though predominantly seen in reproductive age group, they may also be seen in children and postmenopausal women. Torsion is the most common complication of this tumour. Age of the patient and the size of the tumour are important prognostic factors in terms of malignant transformation of teratomas. Since 8.9% of these tumours were bilateral it is essential to thoroughly examine the contralateral ovary to rule out bilaterality. Early diagnosis and early treatment is essential to preserve fertility in these patients.

Cutaneous basal cell carcinoma with mixed histology: Cytomorphological features of two unusual cases.

Cutaneous basal cell carcinoma (BCC) is a slow growing locally aggressive malignant tumor. It is usually diagnosed on histopathological examination of the excised biopsy. Recently, fine needle aspiration cytology (FNAC) is emerging as a simple alternative technique for rapid diagnostic work of nodular and plaque-like skin lesions. We report the cytomorphological features of two cases of cutaneous BCC having unusual clinical presentation and mixed histology (MH); emphasizing the diagnostic difficulties encountered on cytology, the plausible explanation and the precautions to keep in mind to avoid misdiagnosis.

Correlation of FOXL2 with Inhibin and Calretinin in the Diagnosis of Ovarian Sex Cord Stromal Tumors.

OBJECTIVE: Alpha-inhibin and calretinin have been traditionally used as immunomarkers for sex cord stromal tumors. However, the variation in their immunoreactivity and their lack of specificity for sex cord stromal tumor makes the search for a more sensitive and specific immunohistochemical marker essential in routine diagnosis of sex cord stromal tumor. This study was conducted to correlate the diagnostic utility of FOXL2 with inhibin and calretinin in the diagnosis of sex cord stromal tumors of ovary. MATERIAL AND METHOD: The study was conducted in the department of pathology. 31 cases of sex cord tumors received in past eight years (2002-2010) were included in this study. Immunostaining for inhibin, calretinin and FOXL2 was performed and compared on the basis of staining intensity and percentage positivity on all the cases. RESULTS: Calretinin was found to be positive in 29/31 sex cord stromal tumors with variable intensities and was negative in two cases of sex cord stromal tumors, inhibin was positive in 28/31 and only three cases had no cytoplasmic staining. All the 31 cases included in this study were positive for FOXL2 with variable staining intensities and percentage positivity. Ten cases of each surface epithelial and germ cell tumors were also negatively stained with FOXL2. CONCLUSION: In contrast to inhibin and calretinin, FOXL2 had a sensitivity and specificity of 100% for all the cases of sex cord stromal tumors included in this study.

Lymphoepithelioma-like carcinoma of cervix: Cytological Features on Conventional Cervical Smear.

Lymphoepithelioma-like carcinoma (LELC) is a rare neoplasm of the cervix. The importance of distinguishing this undifferentiated carcinoma with a predominant lymphocytic infiltrate lies in the fact that despite being poorly differentiated they have a better prognosis. The diagnosis however becomes more challenging when the pathologist is provided with a small cervical biopsy or a Papanicolaou smear. While the reports describing histology and their relation to Epstein-Barr virus (EBV) are many, there are only few case reports describing the cytology of these tumors. We describe the cytological features of LELC of cervix on conventional smear and correlate it with the histopathological findings of the same. A 67-year-old multiparous Hindu woman presented to the gynecology outpatient department with the history of postmenopausal bleeding for the past six months. The cytological examination of the cervical smear (Papanicolaou stain) was done followed by cervical and endometrial biopsy. Based on Papanicolaou smear and biopsy suggestive of a poorly differentiated carcinoma a radical hysterectomy with pelvic lymphadenectomy was performed. Hysterectomy specimen showed the morphology of LELC and was then correlated with the cervical smears retrospectively. On review of cytological smears it was seen that the tumor cell clusters had an abundant lymphoid background, which was overlooked earlier. Immunohistochemistry for EBV was negative. We conclude that the presence of undifferentiated tumor cell clusters with ill-defined cell borders and large number of lymphoid cells in the background suggest the diagnosis of LELC on cervical cytology. Diagn. Cytopathol. 2017;45:239-242. © 2016 Wiley Periodicals, Inc.

Xantogranulomatous Salpingo Oophritis, Lessons Learnt: Report of Two Cases With Unusual Presentation.

Xanthogranulomatous inflammation is a rare form of chronic inflammatory response consisting of macrophages, lymphocytes, plasma cells and neutrophils too. Due to its locally destructive nature and mass forming capacity as a result of adhesions, this type of inflammation may mimic malignancy or tuberculosis both clinically and radiologically. We present a report of two such cases, one mimicking tuberculosis and the other mimicking malignancy clinically. Awareness of this condition and a higher index of suspicion among clinicians, radiologists and pathologists can help in early diagnosis and more appropriate treatment of this potentially destructive disorder.

Spectrum of Childhood and Adolescent Ovarian Tumors in India: 25 Years Experience at a Single Institution.

BACKGROUND: Ovarian tumour in children and adolescent girls form an uncommon but important part of gynaecological malignancies. They account for 1% of all the childhood malignancies and 8% of all abdominal tumours in children. Since the ovarian cysts are thought to arise from mature follicles, these tumours were considered to be infrequent in the paediatric population. AIM: The rarity of this condition prompted us to conduct this study and share our experience on the incidence and clinicopathological features of different ovarian tumours in girls up to 20 years of age observed in last 25 years at a single tertiary care hospital. MATERIAL AND METHODS: This was a retrospective study conducted in the Department of Pathology at a tertiary hospital, Delhi. All ovarian tumours up to the age of 20 years in the past 25 years (1990-2014) were included for the purpose of studying the clinicopathological aspects of ovarian tumours in this age group. Descriptive statistics for prevalence and age-wise prevalence was done. Chi-square test, to find an association between the age, laterality and size with malignancy was performed. RESULTS: We received a total of 1102 cases of ovarian tumours over the period of 25 years (1990 to 2014), of which 112 (10%) cases were seen in girls up to 20 years of age. The mean age of the patients was 15.3 ± 4 years. The most common presenting complaint was pain abdomen (46.4 %) There was a statistically significant correlation found between size and malignancy status of tumours in our study (p = 0.00). Of 112 cases of ovarian tumours, 39/112 (34.8%) were malignant and 73/112 (65.2%) were benign. Mature cystic teratoma (27.6%) was the most common type of benign tumour in this age group and immature teratomas were the most common type of malignant ovarian neoplasms. CONCLUSION: Premenarchal girls with ovarian masses may have varied presentations. Abdominal pain is the most common presenting complaint of young adolescent girls with adnexal masses. So the index of suspicion should be kept high and prompt investigations like ultrasound must be performed early to rule out such adnexal masses. Immature Teratoma was the most common malignant and mature cystic teratoma was the most common benign tumour in our study.

Scrotal leiomyosarcoma associated with bilateral epididymo-orchitis.

Leiomyosarcoma of the scrotum is a rare tumor. We report the case of a 60-year-old man who presented with bilateral testicular enlargement that was clinically misdiagnosed as a testicular tumor. The tumor was excised by bilateral inguinal orchiectomy. Histopathologic examination revealed a scrotal wall leiomyosarcoma with bilateral epididymo-orchitis.