Samir S. Amr

Leiomyosarcoma of the infra-renal inferior vena cava

Leiomyosarcoma of the inferior vena cava (IVC) is a rare slow-growing retroperitoneal tumor. Two percent of leiomyosarcomas are vascular in origin, and tumors of the IVC account for the majority of the cases. The diagnosis is frequently delayed, because affected patients remain asymptomatic for a long period. It has an extremely poor prognosis, with 5-year actuarial malignancy-free survival rates of 30% to 50% after a wide surgical resection. The authors present the case of a patient with IVC leiomyosarcoma who underwent en bloc resection of the tumor along with the involved segment of the infrarenal IVC without caval reconstruction. Complete surgical resection offers the only potential of long-term survival, but survival of unresected patients is generally measured in months. Palliative resections may temporarily improve symptoms but do not offer long-term survival.

Comparing BRAF mutation status in matched primary and metastatic cutaneous melanomas: Implications on optimized targeted therapy

BACKGROUND Selective BRAF inhibitors have shown dramatic results with regard to improving outcome in patients with melanoma. Testing the BRAF status in matched primary and metastatic melanomas to optimize individual targeted therapy is not well investigated. METHODS Extended BRAF testing using PCR for 9 mutations and VE1 immunohistochemistry for BRAF V600E detection on 95 lesions including 40 primary melanomas with their matched metastases (n = 42), recurrences (n = 9) and second primaries (n = 4) was performed. Nine patients had multiple metastases. RESULTS V600E was the only identified mutation type; 35.4% of primary vs. 18.9% of metastatic melanomas. The overall primary-metastatic BRAF status discordance rate was 32.3% using PCR and 27.5% with immunohistochemistry, and was significantly more frequent in primary lesions with mutant BRAF (67%). Males and patients with metastasis to lymph nodes were less likely to be discordant compared to females and those with metastasis to other sites (p = 0.023). Discordant BRAF mutation status was predicted by multivariate binary logistic regression: the presence of a mutant BRAF in the primary melanoma [OR (95% C.I.) = 23.4 (2.4-229.7)] and female gender [OR = 10.6 (1.08-95)]. Inter-metastases BRAF concordance was 100% (6 comparisons). CONCLUSION A high discordant rate implies the need for clinical trials addressing the response to targeted therapy in patients with discordant BRAF statuses between their primary and metastatic lesions.

Strongyloides stercoralis infection in kidney transplant recipients.

Strongyloides stercoralis is an uncommon infection in Saudi Arabia. It can establish latency and cause an autoinfection in humans that lasts for years. The infection can get reactivated during immunosuppression and can result in a life-threatening Strongyloides hyperinfection syndrome. We present three cases of renal transplant recipients who developed Strongyloides infection following transplantation. A bronchoalveolar lavage specimen, a duodenal biopsy and/or a stool specimen from these patients revealed evidence of S. stercoralis larvae. The first two patients received kidneys from the same deceased donor, a native of Bangladesh, an area that is highly endemic for S. stercoralis. The data suggest that the first two cases might be donor derived. High-risk donors and recipients should be screened for Strongyloides infection to initiate treatment before transplantation thus reducing morbidity and mortality.

Role of Surgery After Chemotherapy in B-Cell Lymphoma of Thymus Causing Airway Compression and Right Ventricle Outflow Tract Obstruction

Primary B-cell lymphoma of thymus is uncommon. We describe the case of a 23-year-old woman with a history of shortness of breath and distended neck veins of 1-month duration. A chest roentgenogram and computed tomographic chest scan of the showed a large anterior mediastinal mass. Tru-cut biopsy revealed primary B-cell lymphoma of thymus. She underwent chemotherapy with no symptomatic improvement. Post-chemotherapy computed tomographic scan of the chest showed no appreciable reduction in tumor size. Surgical excision was carried out with excellent results.

Invasive Mucormycosis in a Patient With Liver Cirrhosis: Case Report and Review of the Literature

Introduction Cutaneous Mucormycosis is a rare opportunistic infection caused by Zygomycetes class of fungi that is often fatal, requiring aggressive local control as well as systemic therapy. Few cases of mucormycosis were described in patients with liver cirrhosis, mostly rhino-orbital. To our knowledge, only two cases of upper extremity involvement was reported in cirrhosis while a few cases were reported in the post-transplant setting. We report herein the third case of upper extremity mucor infection in the setting of liver cirrhosis. Case Presentation We described a rare case of forearm infection originating in a traumatic intravenous access portal in a 25 year-old woman with liver cirrhosis secondary to autoimmune hepatitis. Discussion She developed acute on chronic liver failure during the last trimester of pregnancy, which was terminated. Painful, erythematous lesion was noted on her right forearm in the area of intravenous access, which later became necrotic. Extensive debridement was done and histopathological examination confirmed the diagnosis of mucormycosis. The patient started on Amphotericin B. Her condition continued to deteriorate and ended up with above elbow amputation followed by right shoulder disarticulation. She died two days later due to multi-organ failure. In conclusion, forearm mucromycosis in liver cirrhosis can be fatal.

Malignant peripheral nerve sheath tumor with extensive osteosarcomatous and chondrosarcomatous differentiation: A case report

Highlights • MPNST is an aggressive tumor especially in younger age group and even if there is no association with NF1 gene.• Distant metastasis may appear within 1 year of radical R0 resection of the primary tumor.• Exhibition of divergent differentiation may be associated with poor prognosis.• Metastatic MPNST may not exhibit divergent differentiation similar to that exhibited by the primary tumor. This interesting finding warrants further research and analysis to establish its impact on the prognosis.

CD10 and CD138 can be expressed in giant cell tumor of bone: An immunohistochemical study.

Giant cell tumor of bone (GCTB) is a primary bone neoplasm which is characterized by the presence of mononuclear cells (MCs) and osteoclast-like multinucleated giant cells (MNGCs). Up to our knowledge, CD10 immunoreactivity in GCTB has not yet been studied, and only one study touched on CD138 immunoreactivity in GCTB. The objective of this study is to investigate the immunoreactivity of CD10 and CD138 in GCTB. We offer a discussion of our findings in the context of the differential diagnosis, particularly in small biopsy material. We retrieved and reviewed 15 well-documented cases of GCTB from January 2008 to December 2014. Well-controlled standard immunohistochemical satins were performed on these cases for CD10 and CD138 and few other selected antibodies. Immunoreactivity for CD10 was membranous and was found in 14 (93%) cases. This immunoreactivity was found only in the MCs, whereas the MNGC were all negative. CD138 showed variable positivity in 11 (73%) while 4 (37%) were completely negative. Similar to CD10, staining for CD138 was only seen in the MC; however, the immunoreactivity was predominantly concentrated in the peri-vascular areas. Most of GCTB cases can show variable immunoreactivity for CD10 and CD138. The aforementioned immune-expression raise the possibility of a role in the pathogenesis of GCTB. Paying attention to this immunoreactivity is recommended when considering the clinical and radiological differential diagnosis, especially in small biopsy specimens.

Unilateral Virginal Breast Hypertrophy in an 11‐year‐old Girl

was also noted as well as an area of necrosis on the medial aspect of the left breast (Fig. 3). Crepitus was also noted in the area of the axilla. The patient was taken emergently to the operating room where she underwent a modified radical mastectomy with sparing of the axilla and chest wall debridement (Fig. 4). Core needle biopsy is a safe, minimally invasive, and accurate procedure in the evaluation of women with suspected breast cancer. The most common complications associated with this procedure are severe bleeding, hematomas, and infections. However, these are fairly rare. In addition, necrotizing infections of the breast are uncommon. Similar to necrotizing infections in other anatomical sites, the presence of tense edema, skin discoloration, blisters/bullae, tissue necrosis and/or crepitus, suggest an advanced stage in the infectious process. When these findings are present, interventions should not be delayed and should include prompt debridement, broad spectrum antibiotics, aggressive resuscitation, repeated debridement when necessary and aggressive nutritional support.

Mediastinal endodermal sinus tumor associated with fatal hemophagocytic syndrome.

The association of endodermal sinus tumor, known also as yolk sac tumor, of the mediastinum with hemophagocytic syndrome is exceedingly rare with only a few cases on record. We report a 24-year-old male who had a large mediastinal germ cell tumor, proven to be an endodermal sinus tumor on biopsy. The patient developed pancytopenia and coagulopathy related to associated hemophagocytic syndrome, with a fatal outcome. A brief review of the relevant literature is presented as well.

Primary squamous cell carcinoma of the gallbladder: Report of a rare neoplasm from the Eastern Province of Saudi Arabia

Highlights • There is paucity in the literature regarding gallbladder cancer in Saudi Arabia.• Only 3% of gallbladder cancers worldwide are primary squamous cell carcinoma (SCC).• We present the first case of primary gallbladder SCC reported in Saudi Arabia.• Physicians should be aware of this cancer even if it is rare in our country.• We should consider gallbladder cancer when diagnosing gallbladder-related symptoms.