Primary Ovarian Non-Hodgkin’s Lymphoma: Retrospective Study of 16 Patients

Background: Primary non-Hodgkin’s lymphoma (PONHL) of the ovary is rare disease. Ovarian involvement by NHL is usually secondary, as a part of systemic disease. It is frequently diagnosed as ovarian carcinoma which causes a significant delay in diagnosis and management. Objective: To analyze, to report and to better understand the clinico-pathologic features and results of treatment, and prognostic factors of these tumors. Material & methods: This was a retrospective single institutional work that included 16 cases of primary ovarian non-Hodgkin lymphoma (PONHL) treated in National Cancer Institute-Cairo University from January 2010 till January 2015. All available medical data including the clinical and pathological characteristics, treatment, and outcomes of patients with PONHL are analyzed. Results: Data from 16 patients are obtained. The patient’s age ranges from 14 to 55 years (mean 28 years). Ascites is the most common manifestation (75%). Tumor size ranges from 5 to 24 cm (mean 13.1). LDH is elevated in all cases (mean 644 U/L) and CA-125 is elevated in only 4 cases (25%) especially when there is an extensive peritoneal irritation. Ten cases (62.5%) are bilateral with stage IV-E according to the Ann Arbor staging system. The remaining six cases (37.5%) are unilateral with Ann Arbor stage I-E. There are no stage II-E or III-E in the current study. Tumors are classified according to the World Health Organization as follows: diffuse large B-cell lymphoma (10 cases) (62.5%), Burkitt’s lymphoma (5 cases) (31%) and only one case of B-lymphoblastic lymphoma/leukemia. All the tumors are of B-cell lineage and are all CD20 positive. All Burkitt’s lymphoma cases show higher Ki67 index (4 cases are 100% and one is 88%). The case of B-lymphoblastic lymphoma/leukemia is positive for TDT & CD 10. Surgery is the main treatment modality for primary diagnosis and for staging, although chemotherapy should have been the primary treatment because it is one of the most chemosensitive tumors. Follow-up period ranges from 3 months to 5 years (mean 33 months). Ten patients are alive without disease. Two cases experienced relapse and one case died during chemotherapy treatment. The remaining three cases died from other causes than disease. The median overall survival time was not reached yet;however, the mean overall survival was 46.8 monthes;median progression free survival was 36 months. Conclusion: Most patients with PONHL present with symptoms attributable to an ovarian mass which necessitates extensive surgical staging that is not mandatory for lymphomas. More studies will be needed to better define and treat this rare entity.

Yolk Sac Tumor of the Ovary in 18 Egyptian Cases: Does It Really Differ?

Background: Ovarian Yolk sac tumor (OYST) is a rare entity of malignant ovarian germ cell tumors (MOGCT). Abdominal pain, a rapidly growing distending mass or irregular vaginal bleeding is the main presentation. Serum AFP is elevated in nearly all cases. The standard management is fertility preserving surgery with adjuvant chemotherapy. Aim of Work: To report and analyze retrospectively recorded cases that were either treated at National Cancer Institute/Egypt or referred there for advice about therapy. Materials and Methods: This is a retrospective single-institutional analysis of 18 cases of OYST treated at National Cancer Institute-Cairo University from January 2011 till December 2015. The clinical and pathological characteristics, treatment, and outcomes of these patients were analyzed. Results: Data from eighteen patients were obtained. The median age was 18 years (range: 15 - 22). Abdominal pain was the most common presentation (89%). The mean tumor size was 21cm (range: 8 - 30 cm). Eleven of our cases (61%) were stage I, seven cases and (39%) were stage IV at presentation. Fifteen cases (83%) underwent fertility preserving procedure & the standard surgical staging. Panhysterectomy & formal staging procedure was done only in two cases (11%). One case (6%) underwent bilateral salpingo-oophorectomy. 2 cases (11.1%) only underwent lymph node biopsy. 11 patient (61.1%) showed pure type YST while mixed type was present in the remaining 7 cases (38.8%): Dysgerminoma (one case, 5.6%), Dysgerminoma + immature teratoma (one case, 5.6%), Immature teratoma (2 cases, 11.1%) and Teratoma (3 cases, 16.7%). AFP was extremely elevated in all cases at presentation (median 4191 ng/mL;ranging: 725 ng/mL - 402,908 ng/mL). It showed decreased level after surgery (median 145 ng/ mL;ranging: 2 ng/mL - 38,000 ng/mL) & normalized after chemotherapy except for progressive disease. All cases started BEP regimen after surgery with complete remission in twelve cases. In follow up period (median 17 months;ranging: 2 - 48 months) two patients relapsed;the mean overall survival time was 34.2 and progression free survival was 33.84 months respectively. Conclusion: Ovarian yolk sac tumors are rare neoplasms. Surgery with adjuvant chemotherapy is the standard management. AFP is important for diagnosis, monitoring response to treatment and predicting relapse.

Granulosa Cell Tumors of the Ovary: Retrospective Analysis of 17 Cases

Background: Granulosa cell tumors (GCTs) are rare neoplasms with a relatively favorable prognosis. They are characterized by a prolonged history and a tendency to late recurrences. It is the most common type of sex cord-stromal tumors. Aims: To analyze, to report and to better understand the clinico-pathologic features and results of treatment, and prognostic factors of these tumors. Materials and Methods: A retrospective single-institutional review 17 cases of GCTs were treated in National Cancer Institute—Cairo University from January 2010 till December 2014. The clinical and pathological characteristics, treatment, and outcomes of patients with ovarian GCTs were analyzed. Results: Data from 17 patients were obtained. The median age was 54 years (range;14 - 72). Abdominal pain was the most common presentation (64.7%). The mean tumor size was 14 cm (range;7 - 23 cm). The majority of our patients were stage I (n = 11;64.7%), while (n = 3;17.6%) had stage III and (n = 2, 11.8%) were stage IV. Only one case (5.9%) had an unknown stage (explored outside NCI). The majority of cases were of adult type disease (n = 14) and low grade pathology (n = 10). In follow-up period (median = 42 months;ranging 9 - 60) three patients relapsed;the median overall survival time was not reached yet, however, the estimated 3-year survival was 72.5%. Conclusion: Granulosa cell tumors are rare neoplasms of the ovaries. They progress slowly and often are diagnosed in an early stage. Surgery is the main line of treatment. Prolonged post-therapeutic follow-up is necessary. Definition of proper prognostic factors is mandatory.

Management of Thymic Neoplasms: Egyptian NCI Experience

Background: Thymic tumors are rare entity with little information regarding outcomes after therapy with curative intent. They are heterogeneous group of neoplastic lesions with wide spectrum of morphologic appearances. They show different presentations with a variable & unpredictable evolution ranging from indolent attitude to highly infiltrative and metastasizing one. The optimal treatment includes surgical resection, chemotherapy, and radiotherapy. Objectives: To analyze, to report & to better understand the clinico-pathologic features, results of treatment, and prognostic factors of these tumors. Materials and Methods: From 2008 to 2014, 13 patients (8 men, 5 women) underwent surgical resection of thymic tumor at a mean age of 47 years. Patient demographics, extent of surgical resection, and outcomes were compiled. Demographic variables, use of chemotherapy or radiotherapy, peri-operative variables, recurrence rates, and long term survival were analyzed retrospectively. The Masaoka stage and tumor diameter were recorded along with other variables that potentially influenced survival such tumor grade, site & number of metastatic disease. Results: The mean age of our patients was 47 years (range: 21 to 58 years) & 8 cases (61.5%) were men. The distribution of patients according to the Masaoka staging at presentation was, stage I (6 cases, 47%), stage II (3 cases, 23%), stage III in (1 patient, 7%), and stage IV (3 cases, 23%). Neoadjuvant chemotherapy was administered to 3 patients (23%) whose tumors were deemed to be more locally advanced precluding R0 resection from the start & 2 patients (15%) received neoadjuvant concomitant chemo-radiotherapy. No patient demonstrated an associated immunologic disorder such as myasthenia gravis. In all patients (13 cases, 100%) pathologic confirmation of thymic tumor was by CT guided fine needle aspiration/biopsy. Surgical resection was done for all our cases (13 patients, 100%) through median sternotomy approach in (11 cases, 86%), cervical approach (1 patient, 7%) & transverse sternotomy with clamshell incision in 1 patient (7%) with a large thymic carcinoma whose postoperative pathology revealed a close margin of resection (R1). Resection of nearby structures was done in our cohort to fulfill R0 resection;pulmonary wedge resection in (2 cases, 15%), pleural resection in (5 cases, 38%), lobectomy in (1 patient, 7%) & unilateral phrenic nerve resection was performed in (2 patients, 15%). None patients with phrenic nerve injury underwent diaphragmatic plication to improve respiratory insufficiency No superior vena cava resection was done in any of our cases. There were no perioperative deaths, tracheostomy, or postoperative respiratory failure occurred. Complete R0 resection was achieved in 12 cases (92.3%), and of these patients, only 1 patient (7.7%) experienced local recurrence whose primary resection was R1. 4 patients received adjuvant chemotherapy or radiotherapy or both. Mean duration of survival in the entire group was 22.7 months (range: 14 to 36 months). At the last follow-up, (8 patients, 61.5%) were alive without disease, (1 case, 7.5%) was alive with disease, and (4 cases, 31%) were dead. Conclusions: Thymic tumors are amenable to surgical therapy, with increased use of computed tomography imaging. Patients with early stage disease are being identified more frequently. Complete surgical resection appears to have favorable cure rates in these patients. Patients with locally advanced disease can experience long term survival with a multimodality approach.

Extracranial Head and Neck, Peripheral Nerve Tumors: A 10-Year Experience at NCI, Egypt

Background: Peripheral nerve tumors (PNT) originate from the neural crest and typically formed from Schwann cell sheath. Considering the number of nerves in this area, they form a very small percentage of neoplastic lesions of the head & neck. A solitary mass in the lateral neck frequently confronts the surgeon. An uncommon cause for such a nodule is a neoplasm arising from nerve tissue with PNTs being the commonest of them to face. Aims: To study the clinico-pathological characteristics, presentations, surgical approaches, postoperative complications & outcome of management. Materials & Methods: a single institution combined retrospective & prospective study. A retrospective analysis of all cases presented NCI-Cairo University with H&N extracranial PNST candidate for surgery from Jan 2009 until the end of Dec 2015 & prospective analysis of all cases with the same presentation presented to the NCI from Jan 2016 until the end of June 2019. 23 cases were included. Data were collected then analyzed. Results: Mean age was 34.7 years with female to male ratio 1.9:1. Mean size was 5.4 cm. Commonest presentation was asymptomatic neck lump located laterally in the neck (9 cases, 39%). CT scanning was done in 17 cases (74%). As regard pathology, we have only 4 malignant cases (17%). Most tumors (9 cases, 39%) originated from cranial nerves. 18 cases (78%) were resected through the neck. Complication occurred in 13 cases (56%), most of them during tumor dissection from its nerve origin or root. Vascular complications occurred in 6 cases (26%) due to bleeding controlled with ECA ligation (facial ischemia, hemiparesis & paresthesia). Cranial nerve injury (XI, XII) occurred in two cases (8.7%) & Speech defects in other two 2 cases (8.7%) due to maxillary resection. Postoperative?radiation was given to all 4 malignant cases (17%) of MPNST with Postoperative Chemotherapy to all cases experienced relapse after adjuvant RT. Post-operative palliative RT was given to (2 cases, 8.7%) with bone deposits. Conclusion: The vast majority of H&N extracranial PNTs are benign. Proper diagnosis is mandatory for ideal treatment. Complete R0 resection may not always be justified, therefore intracapsular or debulking procedures may have a role. The Malignant counterpart of PNTs (MPNST) is highly aggressive. They are treated with multimodality approach involving surgery with CTH & RT.