Intra-abdominal cystic lymphangioma in adults: A case series of 32 patients and literature review.

Introduction: Cystic lymphangioma (CL) is a benign tumor originating from the lymph vessels. Lymphangiomas in the abdominal cavity are extremely rare, particularly in adults.This article was designed to study the epidemiological, diagnostic difficulties, and therapeutic principles of intra-abdominal cystic lymphangioma (ACL) in adults. Material and methods: We conducted a single-center, retrospective study of 32 adult patients with ACL admitted to surgical department "A" in "La Rabta Hospital" in Tunis, from January 1998 through December 2020. The demographic, clinical, biological, radiological characteristics, histopathologic, and therapeutic data were collected, as well as the surgical intervention used and the postoperative immediate and late complications. Results: Thirty-two adult patients with ACL were recruited, including 20 females and 12 males. The median age at treatment was 47 (range 14-80) years. The most prevalent sites were the retroperitoneum (25%), the mesentery (21.9%), and the paracolic gutters (n = 18. 7%). Twenty patients underwent open surgery (62.5%), whereas 12 cases (37.5%) had laparoscopic surgery. Twenty-eight patients received total cystectomy (87%). Three recurrences were observed during follow-up (9.4%). Conclusion: The clinical features of CL in adults remain unclear. The diagnosis is only confirmed by histopathological examination after complete surgical resection. The laparoscopic approach is considered safe and feasible.

Clinical and pathological characteristics, treatment outcome and prognostic factors in adult rhabdomyosarcoma: a monocentric retrospective study.

Rhabdomyosarcoma (RMS) is rare in adults. Our study is the first in Tunisia to report outcomes of adult RMS. We retrospectively analyzed clinical data of adult RMS patients. We collected data regarding clinical characteristics, treatment outcome and prognostic factors. Survival was assessed using the Kaplan Meier method. Forty-seven patients were included. Median age was 39. Twenty-five patients were young adults (53%). Sex ratio (M/F) was 1.9. RMS was localized in 33 patients (70%) and metastatic in 14 patients (30%). Extremities were the most frequent tumor site (40%) followed by trunk (23%). Median tumor size was 9 cm. Pleomorphic RMS was the major subtype (36%). Twenty seven of 33 patients with localized RMS underwent surgery (82%). Relapse free survival (RFS) was 38%. Young adults had a significantly worse RFS than adults aged ≥40 (p = 0.045). Surgery was associated with a significantly better RFS (p = 0.023). Five year overall survival (OS) was 35% and 27% in localized and metastatic RMS respectively. RMS localized in the extremities had significantly poorer OS (p = 0.041), same as non-operated patients (p = 0.025). OS for metastatic RMS was significantly worse after surgery of the primary tumor (p = 0.002). In multivariate analysis, surgery (HR = 0.108; 95%CI (0.023-0.519); p = 0.005) and non-extremity localization (HR = 0.238; 95%CI (0.075-0.751); p = 0.014) were independent prognostic factors for OS in localized RMS. Adults with RMS have poor 5 year OS. Surgery and non-extremity localization were independent prognostic factors for OS in localized RMS.

Clinico-Epidemiological Profile, Prognostic Factors and Treatment Outcome of Refractory Hodgkin Lymphoma.

Approximately 5-10% of patients with Hodgkin lymphoma are refractory to initial treatment. The aim of our study was to assess the clinico-epidemiological profile, prognostic factors and treatment outcome. A retrospective study was conducted over a period of 12 years between June 2006 and January 2018 at the oncology department of Salah Azaïz Institute. Thirty-one patients were included. The median age was 27 years with a female predominance (sex ratio = 0.93).The majority had an advanced stage (61%). IGEV regimen was the most commonly used salvage chemotherapy (n = 14). Age above 30 years was predictive of treatment failure after salvage therapy (p = 0.003). IGEV regimen showed better results than ICE protocol in terms of response to salvage therapy (p = 0.048). Seven patients had salvage radiotherapy. Four patients had autologous stem cell transplant. Progressive disease (n = 12) was the main cause of non-eligibility of autologous stem cell tansplant. Overall survival and progression free survival at 3 years were 50% and 5% respectively. The prognostic factors influencing the overall survival were age above 30 years (p = 0.001), advanced Ann Arbor stage before progression (p = 0.02), advanced Ann Arbor stage of refractory Hodgkin lymphoma (p = 0.001), histological subtype (p = 0.001), CD20 expression (p = 0.027) and non-response to salvage therapy (p = 0.004). The prognostic factor influencing progression free survival was the non-response to salvage therapy (p = 0.045). The prognosis of refractory Hodgkin lymphoma remains poor. The current standard secondary treatment consists of combination therapy, usually followed by autologous stem cell transplantat. Innovative therapies are needed to improve the prognosis of refractory Hodgkin lymphoma. Supplementary Information: The online version contains supplementary material available at 10.1007/s12288-021-01463-4.

Petrous metastasis of a lung small cell carcinoma.

Malignant tumors of the temporal bone are rare. They include a wide histological variety. They are mostly primary tumors with an estimated incidence of 0.8-1.0 per 1,000,000 inhabitants per year [1]. Metastases form an uncommon subgroup. The clinical features of these temporal bone metastases are nonspecific and predominantly consist of hearing loss, vertigo, facial palsy, tinnitus, headache, otalgia or otorrhoea [2]. The aim of our publication is to report a rare case of metastasis in the temporal bone as initial manifestation of lung cancer. Our patient was a 44-year-old man who presented for a right mastoid swelling with a grade VI right facial nerve palsy. Imaging and biopsy confirmed the diagnosis of small cell lung carcinoma petrous metastasis. The treatment was palliative. An osteolytic lesion of the temporal bone in an elderly patient may fit with a primary tumor of the temporal bone. It must, also, suggest the diagnosis of metastasis. The malignant tumor most frequently responsible is breast cancer, but lung cancer must also be considered in smoking patients.

Prognostic factors in High-Grade Localized Osteosarcoma of the Extremities: The Tunisian Experience.

BACKGROUND: Osteosarcoma is the most frequent bone cancer occurring in children and adolescents aged 10-20 years. Several prognostic factors have been identified by studies done at western centers. The aim of our study was to identify the prognostic factors in Tunisian patients in order to improve their management. METHODS: We reviewed the data of localized limb osteosarcoma patients treated in Salah Azaïz Institute from January 1980 to December 2018. Patient's treatment and survival variables were assessed. Patients received a neoadjuvant chemotherapy and underwent surgery in an expert center. They received afterward an adjuvant chemotherapy depending on the tumor necrosis assessed by Huvos. RESULTS: Eighty-five patients were enrolled. Mean duration of follow-up was 30 months (range 1-297 months). Males were 1.6 times more frequent, median age was 17 (from 1 to 62 years). Conventional osteoblastic osteosarcoma was the most frequent histological subtype (77%). Median tumor size was 10 cm. Femoral location was the most frequent (60%). The overall average history of symptoms was 103 days (4 to 423 days). The 5-year overall-survival was 38% and the event free survival 32%. Tumor site, lactate dehydrogenase levels, high methotrexate levels at 24 h, clinical evaluation of the tumor perimeter, surgery type and delay of relapse were found to affect overall survival. Tumor site, Lactate dehydrogenase levels and clinical evaluation of the tumor perimeter affected the progression free survival. CONCLUSION: Demographic characteristics of Tunisian patients are mainly the same than worldwide. Femoral site, normal level of lactate dehydrogenase, a clinical response during neoadjuvant treatment, an R0 surgery, a delay of relapse over 2 years and Median H24 Methotrexate level superior to 4.4 µmol/l were associated with a better prognosis in our study.

Suggestion of Tunisia's medical oncologist in the management of breast cancer during COVID-19 pandemic.

Cancer patients are at higher risk to be infected with COVID-19 and to develop a more severe form. Breast cancer (BC) treatments, including chemotherapy (CT), targeted therapy and immunotherapy can weaken the immune system and possibly cause lung problems. For all these reasons Salah Azaiez Institute's department of Medical Oncology took drastic actions to protect patients. In this article we will discuss protocol adjustments taken during the COVID-19 pandemic for breast cancer patients.

The Management of Desmoid Tumors: A Retrospective Study of 30 Cases.

OBJECTIVES: Desmoid tumor also called aggressive fibromatosis is a rare type of benign tumor. It is a mesenchymal malignancy without metastatic potential. The standard management is resection, but other options including observation may be discussed. Desmoid-type fibromatosis may occur throughout the body, but the abdominal wall is the most common site. The aim of our study was to assess the clinicoepidemiological profile, prognostic factors, and treatment outcome of desmoid tumors. METHODS: A monocentric retrospective study was conducted over a period of 19 years between February 2000 and November 2019 at the oncology department of Salah Azaïz Institute. Our study concerns 30 patients with desmoid tumor. All data regarding patients were obtained from the medical record. RESULTS: Thirty patients were included. The median age was 35 years with a female predominance (sex ratio = 0.07). A palpable mass was the most common complaint (n = 27). Median tumor size was 5 cm. The principal site of involvement was the abdominal wall (n = 14). Surgery was performed in 27 patients. The histopathology reports listed 14 (52%) cases with negative margins and 13 (48%) cases with positive margins. Radiation therapy was performed in 2 patients. One patient received tamoxifen. Local recurrence occurred in 11 patients. Two patients died of their desmoid tumor. Abdominal wall tumors have less risk of recurrence compared with other sites (p=0.047). Macroscopic margin involvement (R2) was the only prognostic factor influencing disease-free-survival (p=0.034). CONCLUSION: Desmoid tumors are aggressive tumors with a tendency for local recurrence. Abdominal wall tumors have less risk of recurrence. Macroscopic margin involvement was the only prognostic factor that affects disease-free-survival.

Subcutaneous Panniculitis-Like T-Cell Lymphoma Presenting with Full Facial Edema and Hemophagocytic Syndrome.

A 42-year-old previously healthy woman presented with a 2-month history of recurrent fever and painful swelling on the left thigh. She was given a presumptive diagnosis of cellulitis and an antimicrobial. Because the response was not significant and fever remained moderate to high grade, with the appearance of gradually increasing periorbital edema (Figure 1), the diagnosis was reconsidered, and she was referred to a tertiary referral center for further study.

Gestational trophoblastic neoplasia: experience at Salah Azaiez Institute.

Gestational trophoblastic disease (GTD) develops from abnormal cellular proliferation of trophoblasts following fertilization. It includes benign trophoblastic disease (hydatidiform moles (HM)) and the malignant trophoblastic diseases or gestational trophoblastic neoplasia (GTN). The frequency of the GTD in Tunisia is one per 918 deliveries. The aim of this study is to analyze the clinical characteristics, treatment and outcomes of GTD at Salah Azaiez Institute (ISA). Medical records of women diagnosed with GTD at ISA from January 1st, 1981 to December 31st, 2012 were retrospectively reviewed. FIGO score was determined retrospectively for patients treated before 2002. One hundred and nine patients with GTN were included. Patients presented with metastases at 43% of cases. The most common metastatic sites were lung (30%) and vagina (13%). Fifty six (56 (51%) patients had low-risk and 21 (19%) cases had high-risk, the FIGO score was not assessed in 32 cases. After a median follow-up of 46 months, 21 patients were lost to follow-up, 12 patients died, 19 progressed and 8 relapsed. At 10 years, the OS rate was 85% and the PFS rate 79%. OS was significantly influenced by the presence of metastases at presentation (M0 100 % vs. Metastatic 62 %; p < 0.0001), FIGO stage (I-II 100% VS 61% and 65% for stage III and IV; p < 0.001), FIGO score (low-risk 99 % vs. high-risk 78 %; p < 0.001). GTN is a significant source of maternal morbidity with increased risk of mortality from complications if not detected early and treated promptly.

[Synchronous pulmonary and lachrymal glands involvement by MALT lymphoma]. / Atteinte synchrone pulmonaire et des glandes lacrymales par un lymphome de type MALT.

Primary lung lymphomas are rare tumors representing less than 1% of malignant tumors of the lung. The most frequent form is the mucosa-associated lymphoid tissue (MALT). Ocular involvement is also rare and it is mostly located in the lachrymal glands. We report the case of a patient with pulmonary MALT lymphoma associated with synchronous involvement of the lachrymal glands. This study illustrates the nonspecific clinical, radiological and evolutionary features of this disease.

Cutaneous leiomyosarcoma on the trunk: An unusual presentation with an aggressive course - Case report and review of literature.

Primary cutaneous leiomyosarcoma (PCL) are soft-tissue sarcoma, arising in the dermis, with or without extension into the subcutis. They are thought to have an indolent course compared to their subcutaneous counterparts, they may recur but rarely metastases. We report the case of a patient with a PCL arising in the anterior trunk wall who developed pulmonary, bone and retroperitoneal metastases 6 years after wide surgical excision of the primary tumor.

Potential predictive factors for pathologic complete response after the neoadjuvant treatment of rectal adenocarcinoma: a single center experience.

OBJECTIVE: : To assess the response rate of patients with rectal adenocarcinoma to neoadjuvant therapy and to identify the predictors of histological regression after neoadjuvant radiotherapy (RT) or concurrent chemoradiotherapy (CCRT). METHODS: : This study recruited 64 patients. The patients had resectable cancer of the lower and the middle rectum (T3/T4 and/or N+) without distant metastasis and received neoadjuvant RT or CCRT followed by radical surgery with total mesorectal excision (TME) between January 2006 and December 2011. The patients were classified into non-response (NR), partial response (PR), and pathologic complete response (pCR) based on the Dworak tumor regression grading system. RESULTS: : The median age of patients was 57 years (ranging from 22 to 85). A total of 24 patients were treated with neoadjuvant CCRT, whereas 40 patients were treated with RT alone. Abdominoperineal resection (APR) was performed on 29 patients (45%). Anterior resection with TME was performed on 34 patients (53%). One patient had local resection. Histologically, 12 (19%), 24 (73%), and 28 (44%) patients exhibited pCR, PR, and NR, respectively. Univariate analysis revealed that the predictors of tumor regression were as follows: the absence of lymph node involvement from initial imaging (cN0) (P=0.021); normal initial carcinoembryonic antigen (CEA) level (P=0.01); hemoglobin level ≥12 g/dl (P=0.009); CCRT (P=0.021); and tumor downstaging in imaging (P=0.001). Multivariate analysis showed that the main predictors of pCR were CT combined with neoadjuvant RT, cN0 stage, and tumor regression on imaging. CONCLUSIONS: : Identifying the predictors of pCR following neoadjuvant therapy aids the selection of responsive patients for non-aggressive surgical treatment and possible surveillance.

Primary leiomyosarcoma of thyroid gland: the youngest case.

Primary leiomyosarcomas of the thyroid gland are extremely rare. We report a case of a 32 year-old women with a multinodular goiter. She underwent total thyroidectomy. The tumor histology showed spindle-shaped cells that expressed desmine, caldesmone and smooth muscle actine but were negative cytokeratins.