P11. TESTICULAR EMBRYONAL RHABDOMYOSARCOMA: ITS NEVER TOO LATE
Peiyeing TEOH¹, Fongjuen KIEW¹, Shobana MUKUNDA DEVAN¹, Zahrah TAWIL¹, Rosna YUNUS²
¹Department of Pathology, Hospital Selayang, Selangor.
²Department of Pathology, Hospital Kuala Lumpur, Wilayah Persekutuan Kuala Lumpur.
Rhabdomyosarcoma is the most common soft tissue sarcoma in children. After head and neck, the genitourinary tract is the second most common site, mostly at the paratesticular region. We present a rare case of testicular rhabdomyosarcoma.
A 16-year-old Chinese male presented to Hospital Selayang with the history of painless right testicular swelling for a year. Tumour markers were within normal limits (βhCG<2.0 mIU/ml and αFP1.5 ng/ml), with mildly elevated LDH of 450 u/I. Computed tomography of thorax/ abdomen/ pelvis showed a large heterogenous right testicular mass measuring 197x134x82 mm, with abdominal and pelvic lymphadenopathy, as well as lytic lesions at T2, T5 vertebrae, right sacral alar and left ilium suggestive of bony metastases. Subsequently, he underwent right inguinal orchidectomy. Macroscopic examination revealed a testicular tumour measuring 160x105x67 mm. Cut section showed heterogenous solid tan appearance with myxoid change and areas of necrosis. Microscopically, the tumour was composed of alternating hyper- and hypocellular zones within a loose myxoid stroma. The tumour cells were composed primarily of primitive round to oval hyperchromatic cells with scant cytoplasm. Conspicuous rhabdomyoblastic differentiation was observed. The tumour cells showed patchy immunoreactivity for Vimentin, Desmin, Myogenin and MyoD1. The final diagnosis was embryonal rhabdomyosarcoma, Federation Nationale des Centres de Lutte Contre Le Cancer (FNCLCC) grade 3.
Testicular rhabdomyosarcoma is a rare sarcoma, representing only 7% of rhabdomyosarcoma. Embryonal rhabdomyosarcoma is the predominant histological subtype in 90% of testicular rhabdomyosarcoma. It is a highly aggressive tumour that may present with early metastases. A radical inguinal orchidectomy followed by adjuvant chemotherapy is the recommended treatment.
P12. MULTIPLE UMBILICAL CORD STRICTURES: A CAUSE OF INTRAUTERINE FETAL DEMISE
Mianxin CHEW, Pei Yeing TEOH, Yin Ping WONG, Geok Chin TAN
Department of Pathology, Universiti Kebangsaan Malaysia Medical Centre, Wilayah Persekutuan Kuala Lumpur.
There are multiple types of umbilical cord abnormalities such as short cord, long cord, knots, hyper-coiling, hypo-coiling, single umbilical artery, supernumerary umbilical vessels, cystic and vascular malformations as well as abnormal insertion of cords. Umbilical cord stricture is an uncommon type of umbilical cord abnormalities and has been described to be associated with intrauterine fetal death and a possibility of recurrence.
A 35-year-old woman, para 6+1, delivered a stillborn female fetus at 33 weeks of gestation. There was no previous history of umbilical cord abnormalities. She had no other medical illnesses and there was no history of consanguinity. Her current pregnancy was fairly uneventful until at 30 weeks of gestation, she was found to have polyhydramnios with amniotic fluid index of 35.9 (30 weeks) and 42.9 (31 weeks) during her routine antenatal follow up. A detail ultrasound scan of the fetus at 31 weeks of gestation showed no structural anomaly. She was admitted for close fetal heart rate and cardiotopography monitoring. On day 12 of hospitalisation, there was no fetal heart sound detected and a diagnosis of intrauterine fetal demise was rendered. Postmortem examination showed a macerated fetus weighing 1300 grams with multiple umbilical cord strictures seen at both the fetal end and placental end associated with vascular thrombosis. There was no fetal structural anomaly detected.
Umbilical cord strictures can be associated with intrauterine growth restriction and oligohydramnios. However, polyhydramnios was detected in the present case. The exact aetiology of the mechanism of formation of stricture is still unknown. Umbilical cord stricture is a known cause of intrauterine fetal death yet uncommon. Multiple cord strictures are also possible. Though most of these strictures are located near the fetal end, it can also occur near the placental end.
P13. FROM PROSTATE BIOPSY TO RADICAL PROSTATECTOMY: HOW WELL IS THE CORRELATION? – AN EXPERIENCE WITH 41 CASES IN A TERTIARY CENTER
Zahrah Tawil¹, Teoh Pei Yeing¹, Kiew Fong Juen¹, Wan Azura Wan Yaacob¹
¹Pathology Department, Hospital Selayang, Selangor
Prostate cancer is the most common malignancy in men and the second cause of cancer-related mortality after lung cancer. In this study, we attempt to determine the correlation of Gleason scoring and group grading of biopsies with the final grade of the tumor observed within the corresponding radical prostatectomy specimen in our centre which is one of the referral centres for urological malignancies.
In this cross sectional study, we analyzed the results of prostate needle biopsies and subsequent prostatectomy of confirmed cases of prostate adenocarcinoma diagnosed at the laboratory of Hospital Selayang in 41 patients, between 2014 and 2018. Preoperative clinical data and the information from biopsy and prostatectomy specimens were collected. Pearson and Spearman correlation coefficient were used to determine the strength of the relationship between different variables collected from the patients’ data.
The Gleason scores for the biopsy correlated well with the final Gleason score obtained from the corresponding prostatectomy specimen. This was also in keeping with the overall group grade as well. The Gleason grades were commonly scored at 7 and the corresponding group grade was scored at 2. The Prostatic Specific Antigen levels also correlated well with the final group grade.
There is good correlation between the Gleason scores and group grading of needle biopsies and the final scores obtained from corresponding prostatectomy specimens. To further strengthen this correlation, a larger study involving a larger sample size across a larger duration may be required.
P14. PENILE PARAFFINOMA: A CASE REPORT
Nadzrin binti Md. Yusof / Norhafizah binti Mohtarrudin
University Putra Malaysia
Penile paraffinoma is an uncommon entity associated with paraffin injection into penis. The injection is usually self-induced or performed by non-medical personnel. Injection of liquid paraffin leads to destructive effect to penile structures and functions. The practice is common among Asian and Eastern Europeans for cosmetic, therapeutic or penile girth enhancement. It is a rare entity locally, though has been increased due to migratory fluxes.
A 58-year-old man presented with scrotal swelling and erectile dysfunction for a week. There was no history of fever or discharged. It was associated with penile swelling for a year. He had a history of vaseline injection in the penis 20 years ago. Subsequently he had six penile injections of herbal medicine; the last injection was performed one year ago. On examination, there was a diffuse swelling of penile glans and scrotum measuring 45x40x20mm and 150x35mm respectively. Ultrasound showed numerous tiny sub cm anaechoic foci; the largest measuring 0.2 cm. Routine laboratory investigation was normal. The patient underwent excision of penile glans and scrotal flap. Histologically, penile tissue shows diffuse lipid vacuoles embedded within sclerotic stroma. The vacuoles are varies in size; some are cystically dilated and forming pseudocysts. Granulomatous reactions with abundant foreign body giant cells, foamy macrophages and inflammatory cells infiltrate are seen in the background. Post-operatively, the swellings subsided and the patient resumed erectile and normal sexual functions.
Interstitial exogenous oils lead to granulomatous foreign-body reaction inducing a sclerosing lipogranuloma. Paraffin injection induces many untoward reactions e.g. necrosis, ulceration, infection, fistulization, erectile dysfunction, phimosis and acute urinary retention; as the body lacks the enzymes to metabolize the oils. In this case histological examination is warranted to exclude other differentials such as adenomatoid tumour, lymphangioma or sclerosing liposarcoma as the managements are different.
P15. PLEOMORPHIC DERMAL SARCOMA; A CHALLENGING ENCOUNTER OF A RECURRENT SKIN LESION WITH ATYPICAL CELLS
Siti Shakinah Sobri, Nabilah Huda Hamzah, Haidi Mohamad
Department of Pathology, Hospital Sultanah Bahiyah, Kedah, Malaysia
Pleomorphic dermal sarcoma (PDS) is a rare neoplasm of skin that can be mistaken with atypical dermatofibroma and atypical fibroxanthoma (AFX) in superficial biopsy. Poor margin clearance and recurrence following removal gives a clue to this aggressive tumour.
A 45-year-old man complained of 10 years right nasal alar swelling which had recently increased in size. The lesion was diagnosed as a wart and removed yet it recurred. Clinically, the lesion was 1.5x1cm, smooth, firm, and non-tender. Nasoendoscope displayed healthy nasal mucosa. The biopsy concluded as dermatofibroma with positive margin. The lesion recurred after six months and re-excision was conducted. The initial biopsy comprised of two pieces of skin tissue measured less than 10mm in aggregate diameter. The recurred lesion resembled mushroom-shaped skin tissue, measured 17mm (diameter) x 10mm (length) with presence of scar. Cut section showed a circumscribed, pale yellow subepidermal lesion, measuring 9x7x7mm. Microscopically, both were non-encapsulated dermal tumour composed of spindled to epithelioid tumour cells arranged in haphazard fascicles displaying pleomorphic nuclei with scattered bizarre forms, more noticeable in the subsequent biopsy. Atypical mitoses were identified. Skeletal muscle infiltration was observed. The tumours were positive for SMA, desmin, CD10, CD31, CD68 whilst negative for keratins, caldesmon, myogenin, S100, Melan-A. Ki67 activities were less than 5% and 8-10%, respectively. Follow-up at six months showed no evidence of recurrence.
In view of aggressive clinical behavior and greater pleomorphism with aberrant mitoses encountered, malignancy is a possibility. Expert opinion established a diagnosis of PDS. AFX may represent a precursor of PDS and it is crucial to exclude other atypical spindled cell neoplasms.
P16. THE PREVALENCE OF DIFFUSE LARGE B-CELL LYMPHOMA SUBGROUPS ACCORDING TO HANS ALGORITHM
¹Siti Shakinah Sobri, ¹Nabilah Huda Hamzah, ¹Haidi Mohamad, ²Noor Kaslina Mohd Kornain, ²Nor Salmah Bakar
¹Department of Pathology, Hospital Sultanah Bahiyah (HSB), Kedah, Malaysia, ²Department of Pathology, Faculty of Medicine, Universiti Teknologi MARA, Sungai Buloh Campus, Selangor, Malaysia
Diffuse large B-cell lymphoma (DLBCL) is the most common high grade, non-Hodgkin lymphoma. It can be classified based on morphology, immunophenotyping and genetic expression profiling. Hans algorithm is widely used method to divide DLBCL into two prognostic subgroups; germinal centre B-cell (GCB) and non-germinal centre B-cell (NGCB) using immunohistochemistry (IHC) panels; CD10, BCL6 and MUM1. NGCB patients had been shown to have inferior outcome thus expected lower overall survival. In Asian population, NGCB is more prevalent. This study was conducted to determine the prevalence of DLBCL subgroups in HSB.
Materials and methods:
A retrospective study conducted which assessed 97 cases of DLBCL diagnosed between January 2014 and December 2018. The selected cases were DLBCL (NOS) excluding site-specific subtypes, transformation from low grade lymphomas, concurrent malignancy or HIV and suboptimum tissue. Patients’ demography was retrieved from electronic hospital information system. The haematoxylin-and-eosin stained cell morphology and expression of the IHC panels were examined by three pathologists.
Results and Discussion:
NGCB subgroup of DLBCL predominates by 60.8% (59/97) with the remainder 39.2% (38/97) under GCB subgroup. These findings were similar to data reported by others in Asian countries. Patients’ demography showed 53.6% presented after age of 60. The majority of DLBCL patients were Malay (86.6%), followed by Chinese (12.4%) and Siamese (1%). About 64% (62/97) patients presented with extranodal diseases. The top-three sites for extranodal diseases were head and neck (32.0%), gastrointestinal (16.5%) and genitourinary tract (7.2%). Centroblastic morphology was the commonest microscopic findings (89.7%), followed by anaplastic (4.1%) and immunoblastic (3.1%). Immunohistochemical study of the GCB subgroup showed CD10 expression in 81.6% (31/38). The remaining 18.4% (7/38) showed only BCL6 expression. While, MUM1-positive NGCB cases were observed in 83.0% (49/59) and concomitant BCL6 expression in 64.4% (38/59). Triple negative cases were observed in 17.0% (10/59).
NGCB immunophenotypic subgroup was more prevalent in HSB as seen in other Asian population. Follow-up study to correlate between subgroups of DLBCL and prognosis is suggested.
P17. PRIMARY CUTANEOUS MUCINOUS CARCINOMA OF EYELID: A CASE REPORT
Yusuf Abd Rani, Nor Aizan AA
Pathology Department, Hospital Selayang Selangor, Malaysia
Primary mucinous carcinomas of eyelid was first reported by Lenox et al. in 1951. It’s an exceptionally rare clinical entity with an incidence of 0.07 cases per million individuals. This tumour originates from the sweat glands and majority of this tumour arises on the face with 30% on the eyelids and 43% elsewhere on the head and neck. It usually affects male in 4th to 5th decade. Though it has low metastatic potentials, the recurrence rate is significantly high with incomplete surgical excision. Primary mucinous carcinoma is almost always diagnosed histologically and immunohistochemistry studies are needed for exclusion from metastatic mucinous carcinoma from breast and colon.
We reported a case of 54-year-old gentleman who has been diagnosed with mucinous carcinoma of left lower eyelid in 2016 and recurred after 3 years and presented again in 2019. He initially presented to a private clinic in 2016 with a lump over left lower eyelid for 20 years. MRI brain and orbit showed a subcutaneous lesion (0.9x2.7x1.3cm) with no local extension or involvement of the lacrimal gland. The histological finding from previous and current tumour excision show similar morphology. Immunohistochemistry are positive for CK7, EMA, ER and focally to P63. CK20, CEA and synaptophysin are negative.
Treatment for primary mucinous carcinoma of the skin requires wide local excision with clear margins. It is recognized that distinguishing primary cutaneous adnexal neoplasm from metastatic carcinoma can be difficult and hence organ-specific markers can be used. This case describes how a slow growing lesion on eyelid turns out to be malignant and recurred after incomplete surgical excisions. Surgeon and ophthalmologist should be aware of this tumour in the periocular region and consider this carcinoma in the differential diagnosis of cystic/solid eyelid lesions, even though it appears benign on clinical course.
P18. AN UNUSUAL CASE OF CARDIAC RHABDOMYOMA WITH LEFT VENTRICULAR OUTFLOW OBSTRUCTION.
Fazarina Mohammed¹, Yin Ping Wong², Kan N. Hor, MD³, Michael Arnold, MD, PhD⁴´⁵, Geok Chin Tan, MBBS, PhD²´⁴
¹Department of Laboratory Diagnostic Services, Hospital Canselor Tuanku Muhriz, Universiti Kebangsaan
Malaysia Medical Centre, 56000 Kuala Lumpur, Malaysia
²Department of Pathology, National University of Malaysia, 56000 Kuala Lumpur, Malaysia
³he Heart Center, Nationwide Children’s Hospital and The Ohio State University
⁴Department of Pathology and Laboratory Medicine, Nationwide Children's Hospital, Columbus OH 43205, USA
⁵Department of Pathology, The Ohio State University Wexner Medical Center, Columbus, OH 43210, USA
Although it is the most common paediatric cardiac tumour, cardiac rhabdomyoma is rare in the general population. Diagnosed by echocardiography, surgical intervention is unnecessary unless the patient is haemodynamically compromised, due the tumour’s tendency of regressing spontaneously.
We report a case of an infant born at 38 weeks’ gestation who was found to have a murmur. The infant was born to a 24-year-old mother with uneventful antenatal history and delivery. Both echocardiogram and cardiac MRI showed a large mass at the left ventricular outflow tract, consistent with rhabdomyoma. Echocardiogram at seven weeks of life showed worsening obstruction of the outflow tract. The mass was excised surgically. Microscopic appearance of the mass shows sheets of spider cells with cytoplasmic extensions, confirming the diagnosis of rhabdomyoma. The patient was well post-excision with no disease recurrence to date.
Cardiac rhabdomyoma is regarded as a hamartomatous growth associated with autosomal dominant tuberous sclerosis complex. Investigations and histological appearance of this tumour will be further discussed.
P19. PRIMARY PERITONEAL SEROUS CARCINOMA: A RARE CASE REPORT
Maizaton Atmadini Abdullah¹, Yojenetha Subramaniam²
Department of Pathology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia, Serdang, Selangor1, Department of Pathology, Hospital Serdang, Selangor2.
Primary peritoneal serous carcinoma (PPSC) is a very rare epithelial tumour of mullerian origin. It shares similar clinical, histopathological and immunohistochemical features with epithelial ovarian carcinoma (EOC), however their molecular features are distinct. The diagnosis of PPSC is made when there is intraperitoneal dissemination without ovarian involvements.
We reported a 62 years old multiparous woman presenting with painful abdominal mass and ascites for two months. Abdominopelvic computed tomography (CT) scan revealed gross ascites and extensive omental thickening with multiple small peritoneal nodules. The uterus and both ovaries were atrophic. Lungs were clear. Peritoneal fluid cytology showed atypical cells of undetermined significance with no classical features of lymphoma or adenocarcinoma. A first cycle of chemotherapy was given followed by total abdominal hysterectomy with bilateral salpingo-oophorectomy, omentectomy and appendicectomy. The uterus and both ovaries were atrophic and both fallopian tubes were unremarkable. There were multiple omental nodules seen as well as on the bladder and rectal serosa. Histologically the omental nodules were composed of small sheets and nests of malignant serous cells displaying pleomorphic and hyperchromatic nuclei, coarse chromatin pattern, occasional prominent nucleoli and ample cytoplasm. No glandular formation was seen. They were positive for CK7, WT1, P53 and PAX8. Twenty-four lymph nodes from bilateral pelvic region sampled show no tumour metastasis.
Primary peritoneal serous carcinoma (PPSC) is an uncommon disease with few cases cited in current literatures. PPSC does not have any ovarian involvement, but its histology is that of serous adenocarcinoma. Immunopositivity for mullerian markers are helpful in supporting the diagnosis of PPSC.
P20. PRIMARY SIGNET RING CELL CARCINOMA OF APPENDIX: AN EXTREMELY RARE CASE REPORT
Siti Norhafiza MA, Fauzah Abd Ghani, Razana Mohd Ali
¹Department of Pathology, Hospital Serdang
²Department of Pathology, Faculty of Medicine and Health Sciences, Universiti Putra Malaysia
Appendiceal primary signet ring cell carcinoma (SRCC) is an unusual clinical entity with less than 20 reported cases in literature. It typically affects adults and usually presents clinically with symptoms that are indistinguishable from those of acute appendicitis. SRCC is considered as an aggressive cancer and most patients present with metastases at time of diagnosis.
We report a 19-year-old girl presented with generalized abdominal pain and distention for a week. Both ultrasonographic and computerised tomography (CT) scan done noted heterogenous solid-cystic pelvic mass, with no mention of any appendiceal abnormality. Intraoperative findings showed a right ovarian tumour with omental caking and multiple <1cm nodules on sigmoid colon, pouch of Douglas, and abdominal peritoneal wall. Grossly, the right ovarian tumour is lobulated with intact capsule and solid-cystic tan coloured cut surface measuring 160mm in greatest diameter. The appendix is unremarkable grossly, measuring 8mm in largest diameter. Histopathological examination of the appendix showed multiple foci of malignant cells arising from the mucosa, in areas having diffuse involvement. Malignant cells infiltrations are also seen scattered throughout all layers of the appendix, transmurally up to the serosal layer in singles, small clusters, cribriform pattern and in sheets. The malignant cells exhibit many signet ring cells, having eccentrically placed hyperchromatic nuclei with abundant dusky to eosinophilic cytoplasm. Many small tumourlets are noted within lymphovascular channels. The ovarian tumour is also composed of nests and sheets of signet ring cells with lymphovascular invasion. In view of the diffuse mucosal and transmural involvement of the appendix by SRCC, diagnosis of primary appendix carcinoma with metastasis to the right ovary is made.
As SRCC of appendix is rare, and stomach being a more common site, primary from stomach needs to be excluded. Thus the overall pathology needs to be correlated with the clinical and radiological investigations for correct diagnosis.