Extensive Adenopathy Research Articles

SAT242 Unusual Case Of Sarcoidosis Presenting With Severe Hypercalcemia: Case Report

Abstract Disclosure: G. Bhullar: None. M. Vora: None. Background: This case study discusses an unusual case of hypercalcemia found on routine labs. Clinical Case: A middle-aged Caucasian female with history of MVP, HTN, and anxiety was found to have normal-high calcium of 10.4 mg/dL (nml 8.7-10.4mg/dL) during routine labs offered by her employer. She considered herself relatively asymptomatic and underwent repeat lab testing at her PCP’s office 2 months later which showed calcium of 13.9 mg/dL, ionized calcium of 1.70 mmol/L (nml 1.12-1.32 mmol/L), and intact PTH of 12.4 pg/mL (nml 14.0-72.0 pg/mL). 1 day later, patient presented for an urgent outpatient endocrinology visit. Further questioning revealed polydipsia, polyuria, fatigue, and history of nephrolithiasis more than 10 years ago. Medications included vitamin D, metoprolol, spironolactone, and sertraline. She denied bone pain, weight loss, or GI issues. Physical exam, including heart, chest, and abdomen, was unremarkable. The patient was admitted to a local hospital for hydration and further workup. During her hospital stay, XRAY of chest revealed consolidation in right upper lobe with suspicion of bilateral hilar adenopathy. Contrast CT of chest, abdomen, and pelvis revealed extensive adenopathy, pulmonary nodules, and alveolar consolidation in right upper lobe. Calcified granulomas were found in the spleen. Lab studies showed elevated ACE levels at 87 U/L (nml 14-80 U/L), and elevated activated vitamin D at 108 ng/ml (nml 24.8-81.5 ng/ml). Biopsy results were positive for nonnecrotizing granuloma and negative for malignancy. No suspicious osseous lesions or thyroid gland abnormalities were found. PTHrP and serum protein electrophoresis were found to be in normal range. Hypercalcemia was corrected and patient was treated with glucocorticoids before discharge. Clinical Lesson: Patients with sarcoidosis usually endorse nonspecific symptoms and complaints of pulmonary manifestation may be absent at presentation. 6-13% of sarcoidosis cases involve hypercalcemia (1,2), furthermore, hypercalcemia is more frequently found in white patients (3), who more often present as asymptomatic (4). Thus, clinicians must maintain a high level of suspicion for granulomatous diseases in patients presenting with hypercalcemia regardless of the patient’s symptomology and physical exam findings.

Fatal Case of EBV-negative Posttransplant Lymphoproliferative Disorder With Hemophagocytic Lymphohistiocytosis in an Adult Kidney Transplant Recipient.

Fatal Case of EBV-negative Posttransplant Lymphoproliferative Disorder With Hemophagocytic Lymphohistiocytosis in an Adult Kidney Transplant Recipient.

Not all lymphadenopathy is lymphoma: A case report of Rosai-Dorfman disease

Background: Rosai-Dorfman Disease (RDD) is a rare non-Langerhans cell histiocytic disorder most commonly presenting as bilateral cervical lymphadenopathy in children and young adults. Although a heterogeneous entity with a range of clinical phenotypes, here we describe a presentation at one of the oldest ages found to date in the available literature and its spontaneous resolution in the absence of treatment. Case presentation: A 75-year-old man presented with new-onset right-sided cervical lymphadenopathy and fever, weight loss, fatigue, and anorexia. Physical exam revealed cervical and axillary lymphadenopathy. CT chest/abdomen/pelvis showed extensive adenopathy in the axillary regions, groin, mediastinum, retroperitoneum, splenic hilum, gastrohepatic ligament and porta hepatis, and celiac chains. Left axillary lymph node excisional biopsy revealed sinus histiocytosis with massive lymphadenopathy, consistent with a diagnosis of RDD. Flow cytometry showed mixed polytypic B-cell and T-cell subsets with polyclonal plasma cells. Immunofixation was negative for monoclonal immunoglobulins. On seeing a hematologist one month later, the patient’s symptoms had resolved and he had gained 10+ pounds. Discussion: Rosai-Dorfman Disease is a rare non–Langerhans cell histiocytosis most often seen in children and young adults, although it has been reported up to age 79 years. It typically presents with markedly enlarged, non-tender cervical lymphadenopathy; however, other nodal and extranodal sites can be involved. Due to the rarity of the disease there is a lack of robust data regarding specific treatment modalities, although consensus guidelines were published in 2018 recommending various treatment options, including corticosteroids, chemotherapy, radiation, and surgery. Spontaneous resolution may be observed but can take many months to years. Keywords: sRosai-Dorfman disease; Sinus histiocytosis with massive lymphadenopathy; Non–langerhans cell histiocytosis.

Paracoccidioidomycosis in an Elderly Simulating Cholangiocarcinoma

Paracoccidioidomycosis, the most prevalent deep mycosis in Latin America, is caused by dimorphic species of the genus Paracoccidioides, affects predominantly male rural workers between 30-50 years of age and is an important cause of morbidity and mortality. It can present in two clinical forms: acute/subacute or chronic. In rare cases paracoccidioidomycosis may simulate neoplasia and must be considered as a differential diagnosis of intra-abdominal tumors in endemic areas. We report an exuberant case of subacute paracoccidioidomycosis in an elderly lady, although it is more frequent in the juvenile form. Multiple facial skin lesions were associated with a wasting syndrome and extensive adenopathy including in the hepatic area, causing bile duct dilatation simulating a cholangiocarcinoma. Skin histopathology was diagnostic and antifungal treatment allowed complete resolution of cutaneous and systemic lesions, thus highlighting the merit of the dermatologist, who was fundamental for the conclusive diagnosis and institution of specific therapy, resulting in a favorable outcome.

Customized approach for upfront or delayed resection using radiological criteria in unilateral, nonmetastatic pediatric renal tumors: A prospective study.

The availability of robust, equivalent data regarding outcomes for upfront or delayed surgery for renal tumors in children leads to a dilemma in selecting the initial treatment. Imaging criteria associated with the probability of rupture or incomplete resection may provide a more objective assessment for customization for the timing of surgery. Eighty-three children with unilateral, nonmetastatic renal tumors were enrolled between January 2012 and April 2018. Upfront nephrectomy was performed in the absence or delayed surgery (after a biopsy and chemotherapy) in the presence of one or more imaging-based high-risk features, including perinephric spread or adjacent organ infiltration, tumors crossing the midline, intravascular thrombus, and extensive adenopathy. Post hoc analysis for interobserver concordance for high-risk imaging features was also performed. The upfront surgery group (19) had predominantly stage I or II diseases (89%) and the histological types were Wilms (13), non-Wilms (5) renal tumor, and an inflammatory lesion. The delayed surgery group had 60% with stage I or II diseases and the histological types were Wilms (60) and non-Wilms (4) tumor. In addition, high-risk pathology was identified in nine patients. Overall, 27 patients with Wilms tumors required radiotherapy and anthracycline because of stage III disease, including one in the immediate surgery group. The event-free and overall survival (OS) at a median follow-up of 39 months for Wilms tumor are 88% (95% confidence interval [CI]: 78.5-94.9%) and 89% (95% CI: 81.4-96.6%), 85.1% (95% CI: 73.8-93.4%) and 86.5% (95% CI: 77.4-95.8%) for the delayed, and 100% event-free survival as well as OS (P=.1) in the upfront surgery group. A customized approach pivoted on image-based high-risk features facilitates identification of patients with early-stage renal tumor when the timing of surgery is tailored. Moreover, non-Wilms tumor and high-risk pathology are also identified.

Renal Tumors of Childhood: Radiologic-Pathologic Correlation Part 2. The 2nd Decade: From the Radiologic Pathology Archives.

Malignant renal tumors account for 7% of childhood cancers, and Wilms tumors are by far the most common-but not in older children and adolescents. Among individuals in the latter half of their 2nd decade of life, renal cell carcinoma (RCC) is more common than Wilms tumor. The histopathologic spectrum of RCCs in children differs from that in adults. The most common subtype of RCC in children and adolescents is Xp11.2 translocation RCC, which is distinguished by hyperattenuation at nonenhanced computed tomography, a defined capsule, and associated retroperitoneal lymphadenopathy. Papillary RCC is the second most common histologic subtype. It enhances less intensely compared with the adjacent renal parenchyma and has a propensity for calcification. Clear cell RCC is seen in patients with von Hippel-Lindau disease and is distinguished by its relatively hypervascular nature. Medullary carcinoma affects adolescents with the sickle cell trait and is characterized by an infiltrative growth pattern and extensive metastasis at presentation. Angiomyolipoma is seen in children with tuberous sclerosis complex and is often multifocal and hypervascular, with macroscopic fat. Metanephric tumors are central, circumscribed, and typically calcified. Lymphoma usually manifests as multifocal masses, but it may involve a solitary mass or infiltrative pattern. Extensive adenopathy and involvement of the gastrointestinal tract or other organs also may be seen. Primitive neuroectodermal tumor is an aggressive neoplasm that is typically quite large at diagnosis. Knowledge of the clinical, biologic, and histopathologic features of renal tumors in older children and adolescents and their effects on the imaging appearance can help the radiologist offer a useful preoperative differential diagnosis.

Non-Hodgkinʼs Lymphoma Presenting as Obstructive Jaundice

Non Hodgkin lymphomas are a subtype of lymphomas that consist of diverse group of malignant lymphoproliferative neoplasms. Incidence of obstructive jaundice as an initial presentation of NHL is only seen in 1-2% of patients. We report a unique case of NHL presenting with obstructive jaundice as the initial manifestation. A 36-year-old male presented to the ER with intermittent abdominal pain and jaundice. He also reported nausea, dark urine, night sweats and 20 lbs unintentional weight loss. On physical exam he was tachycardic, jaundiced and tender to palpation in the RUQ and epigastric area. Pertinent labs revealed total Bilirubin of 12.6 mg/dl, Direct bilirubin of 10mg/dl, ALT 224 IU/L, AST 134 IU/L, Alkaline phosphatase 435 IU/L, albumin 3.4 g/dl, Total protein 8.6 g/dl. CT abdomen/pelvis with IV contrast showed a mass obstructing the right and left common hectic ducts, extensive adenopathy in the porta hepatis, necrotic adenopathy adjacent to the pancreatic head, non occlusive thrombus in the superior mesenteric veins and partial thrombus of the right portal vein. The patient was started on heparin drip for the VTE and subsequently had an EUS, which revealed few enlarged lymph nodes in the gastrohepatic ligament. FNA of the nodes was performed with final report showing lymphoid cells with irregular nuclear contours and clumped chromatin positive for CD20, BCL-6 and BCL-2, consistent with diffuse large B-cell lymphoma, germinal center type. Flow cytometry was strongly positive for CD19, CD20 and FMC-7, but negative for CD5, CD10 and CD23 confirming the diagnosis of diffuse large B cell lymphoma. Patient's Total bilirubin continued to trend up during his hospital stay, which resulted in a biliary decompression with percutaneous transhepatic drain. His hospital course was complicated by acute cholangitis treated with antibiotics. Induction chemotherapy with RCHOP was commenced and he was discharged to follow up with oncology outpatient. We plan to follow up with the patient and document his progress. A malignant obstruction of bile duct should alert physicians to the possibility of lymphoma. The presence of jaundice in the context of constitutional symptoms should always raise suspicion of malignant lymphoma and it should be considered in the differential diagnosis of obstructive jaundice.Figure 1Figure 2Figure 3

The Case Files

FigureFigureA 56-year-old black man presented to the ED complaining of face and neck swelling. An initial history was difficult to obtain because the patient did not speak English fluently and had slurred speech. Symptoms started approximately a week earlier when the patient was at his nursing home. He denied dysphagia or odynophagia, he had no rashes, pruritus, dyspnea, or peripheral edema, his face was flushed, and he had visibly-engorged neck veins. He noticed that his tongue was mildly swollen, so he took Benadryl for a week with no relief. The patient said he believed that the symptoms might have been because a nurse gave him an incorrect medication at the nursing home. He had been a pack-a-day smoker for 30 years, and had a history of schizoaffective disorder, bipolar disorder with psychotic features, and GERD. A CBC with differential, PT, PTT, BMP, and UA were all within normal limits. A chest x-ray confirmed a large mediastinal mass, and a soft tissue neck CT scan with contrast found a large mass in the upper mediastinum with extensive adenopathy in the base of the right side of his neck on the right, a right lobe thyroid mass, and occlusion of the superior vena cava and left subclavian vein. The report suggested that the findings were consistent with aggressive lymphoma or lung cancer. The patient was admitted to the hospital with a scheduled emergent biopsy of the neck mass/lymph nodes. He was diagnosed with superior vena cava (SVC) syndrome and small cell lung carcinoma (SCLC) by tissue biopsy and pathology. The SVC drains venous blood from the head, neck, upper extremities, and thorax. Obstruction of the SVC is usually because of neoplasms that invade the tissue wall or put pressure on the SVC. (J Clin Oncol 1984;2[8]:961.) SCLC accounts for 25 percent of SVC syndrome because of malignancy. (HemOnc Today Feb. 10, 2012; http://bit.ly/1H3Uggt.) Clinical features of SVC syndrome include facial edema, difficulty swallowing, engorged neck veins, cough, and dyspnea. (Int J Radiat Oncol Biol Phys 1987;13[4]:531.) Obstruction of the SVC can divert blood flow to the azygous veins, internal mammary veins, superior and inferior epigastric veins, or femoral and vertebral veins, but the venous pressure remains elevated. Complications of this condition include cerebral edema, coma, and death. (Joint Bone Spine 2002;69[4]:416.)FigureThe patient presented with only neck swelling and difficulty speaking. The speech impediment was most likely from the combination of SVC syndrome and right-sided thyroid mass occluding the right recurrent laryngeal nerve. The prognosis of SVC syndrome is poor because of the underlying neoplasm. The patient was promptly started on a dose of approximately 2900 cGy in 13 fractions to the mediastinum and neck nodes and scheduled to receive chemotherapy. After three weeks, he was medically stabilized, and had tolerated chemotherapy and radiation. The SVC syndrome did not compromise his breathing. The patient was advised that he could be discharged with a PORT-A-CATH for future chemotherapy, but the prognosis of his condition remained poor. Lung cancer is the leading cause of cancer-related death in the United States. (CA Cancer J Clin 2008;58[2]:71.) Its incidence is decreasing, but 13 percent of all new cancers are still from lung cancer. (“Lung Cancer Statistics,” American Cancer Society, March 4, 2015; http://bit.ly/1JSd82D.) Even with current screening techniques, approximately half of patients have a metastatic presentation and a 45 percent five-year survival rate. Approximately 90 percent of lung cancer patients are active smokers or had recently stopped. Signs and symptoms of this cancer include shortness of breath, cough, bone pain, weight loss, fatigue, and neurologic dysfunction. (Medscape, March 26, 2014; http://bit.ly/1dPWx29.) None of these were present in the patient. Most SCLCs are metastasized by the time of diagnosis, and are not curative by surgery. A combination of chemotherapy and radiation therapy is required. SCLCs are centrally located masses found in the lung parenchyma. (J Clin Oncol 2006;24[28]:4526.) These pale gray tumors are fusiform-shaped and quite fragile. They can also express neuroendocrine markers and secrete polypeptide hormones that can cause paraneoplastic syndromes (PNS), such as syndrome of inappropriate antidiuretic hormone (SIADH). (Chest 2003;123[1 Suppl]:97S.) The time frame of disease development was unclear, but this patient's presentation was unique because he had nonthreatening symptoms. Lung cancer and SVC syndrome symptoms are usually aggressive, and can compromise the quality of life for patients. Our patient is now being treated to try to prevent the expected disease outcomes.

Gastric and Colonic Mantle Cell Lymphoma With History of Peptic Ulcer Disease

Introduction: Mantle cell lymphoma (MCL) is an aggressive B-cell neoplasm in mantle zone of the lymph node with poor prognosis. There are not many case reports to link between peptic ulcer disease and mantle cell lymphoma. Therefore, we present a case of mantle cell lymphoma in setting of gastric and duodenal ulcers. A 68-year-old Caucasian male presented with chronic anemia from “Goody Powder” use (powder form of aspirin, caffeine, and acetaminophen). The patient had workup with esophagogastroduodenoscopy a year ago with diffuse ulcers in the stomach and the duodenum. Since the diagnosis, he was started on proton pump inhibitor and discontinued aspirin. His esophagogastroduodenoscopy after 8 weeks showed partially healed ulcers. He returned to our office because of weight loss and recurrent iron deficiency anemia without melena or blood in stool. His repeat esophagogastroduodenoscopy revealed congestion in the stomach and non-healing ulcers in the duodenum. His colonoscopy showed abnormal vascularity, erythema, and nodular mucosa of the whole colon. Due to the presence of unusual gastric and colonic appearance, biopsies were sent to tertiary care facility pathology department for expert consultation. Final pathologic examination confirmed MCL from the duodenal and gastric biopsies without H. pylori identified on Giemsa stain. Tissues from the terminal ileum and the whole colon were also tested positive on immunohistochemistry for MCL. His follow-up computed tomography scan of the abdomen and pelvis showed large tissue abnormality in the distal stomach and extensive adenopathy. The patient was referred to oncology and received 6 cycles of rituxan and bendamustine with interval scans showing favorable response. Despite general poor prognosis of MCL, our patient had good clinical response at one year follow up and returned to his baseline with resolution of anemia. Mantle cell lymphoma has an incidence of approximately 4 to 8 cases per million persons per year. The disease occurs in older individuals with a median age around 68 and male predominance. It can present as lymphomatous intestinal polyposis in any region of gastrointestinal tract. To our knowledge, there are limited case reports, which suggest association between mantle cell lymphoma and gastric and duodenal ulcers in absence of H. pylori infection. Therefore, although peptic ulcer disease can be benign especially with history of aspirin intake, we should not exclude the possibility of lymphoma. Edge biopsies should be obtained if the ulcer and mucosa appear to be atypical.

O55 Nodular lymphocyte-predominant Hodgkin's lymphoma (NLPHL): Clinicopathological characteristics and outcome of a rare entity

Twenty-four years after apparently successful treatment for nodular lymphocyte predominant Hodgkin's disease (nLPHD), a 41-year old male developed “B” symptoms and extensive adenopathy. A right axillary lymph node biopsy showed two distinct regions including (1) histiocyte-rich B-cell lymphoma and (2) diffuse small T-cell lymphoma. A clonal rearrangement of the gene for the T-cell receptor beta chain confirmed the presence of a T-cell neoplasm, and this was further confirmed by selective polymerase chain reaction (PCR) on this morphologic zone. PCR on the morphologic B-cell lymphoma confirmed the presence of an immunoglobulin gene rearrangement. These two regions were separated by a less-defined zone containing a mixture of small CD57 positive T lymphocytes, small B lymphocytes, and rare lymphocytic and histiocytic (L&H) cells, highly suggestive of recurrent LPHD. The development of composite B-cell and T-cell lymphoma in this patient raises the speculation that nLPHD may be a neoplasm of lymphoid cells, which can differentiate in both B- and T-cell directions, with the “L&H” cells constituting their B-cell progeny.

X-linked Hyper-IgM Syndrome Associated with Poorly Differentiated Neuroendocrine Tumor Presenting as Obstructive Jaundice Secondary to Extensive Adenopathy

X-Linked Hyper IgM Syndrome (XHIGM) is a rare B-cell immunodeficiency disease. Patients with XHIGM are unable to switch immunoglobulin production from IgM to IgG, IgA, and IgE. Patients with XHIGM require periodic intravenous immune globulin to help prevent infections, and are also at risk for a variety of neoplasms. We describe a young man with XHIGM who presented with obstructive jaundice from malignant adenopathy from widespread, poorly differentiated neuroendocrine tumor. This has not previously been reported and represents a new association with XHIGM.

Follicular Lymphoma of the GI Tract - Distinct Entity or Extranodal Manifestation of Nodal Disease?.

Introduction: Primary GI follicular lymphoma is a rare, poorly studied entity. Current discussions in the literature debate whether or not it is a distinct extranodal entity, similar to cutaneous follicle center lymphoma. Like cutaneous follicle center lymphoma, it appears to have a good prognosis and low incidence of t(14;18).Case History: This is a 74 year old white male who presented for routine screening colonoscopy in May 2003. On evaluation the patient was found to have nodularity of the colon. Biopsies demonstrated several submucosal and mucosal follicles with no distinct mantle zones, composed of a uniform population of centrocytes. No lymphoepithelial lesions or plasma cells were identified. These cells proved to be monoclonal B cells, with a phenotype of CD20+, CD10−/+, bcl-2+/−, bcl-6+, CD5−, CD43−, lambda+ and t(14;18) negative. CD21 stain highlighted the underlying follicular dendritic architecture. No adenopathy was noted at this time. A diagnosis of B cell lymphoma, consistent with extranodal follicular lymphoma, grade I was rendered. As the patient was otherwise asymptomatic, no treatment was given. Four months later, the patient developed retroperitoneal adenopathy and a large pelvic mass. A lymph node biopsy demonstrated numerous large follicles composed of a uniform population of centrocytes, with attenuated to absent mantle zones. The cells were CD20+, CD10+, bcl-2+, lambda+ and t(14;18) positive. There was a comcomitant transitional cell carcinoma of the urinary bladder, which required radical cystectomy. The patient was then initiated on chemotherapy with CVP, for three cycles. He was followed without treatment for 2 years when he developed a tonsillar mass. A biopsy demonstrated a CD10+ diffuse large B-cell lymphoma. Cytogenetic analysis revealed a complex karyotype with a t(14;18). The patient underwent a staging bone marrow, which was positive for follicular lymphoma. The cells were CD20+, CD10−/+, bcl-6+, lambda+, and t(14;18) negative. He was then started on R-CHOP for his B symptoms and extensive adenopathy, and is currently 2 months into therapy.Discussion: This is an unusual case of almost concomitant presentation of t(14;18) negative extranodal follicular lymphoma of the GI tract and classical t(14;18) positive nodal follicular lymphoma. Possible explanations include that these are two manifestations of the same disease, two separate diseases coincidentally occurring around the same time, or a follicular lymphoma clone which has evolved into nodal and extranodal entities.

Intraoperative radiation therapy for high-risk pediatric neuroblastoma

Purpose: To evaluate the efficacy of intraoperative radiation therapy (IORT) in the treatment of high-risk pediatric neuroblastoma. Methods and Materials: Between 1986 and 1998, 23 children received IORT for pediatric neuroblastoma. Electron beam energies ranged from 4 MeV to 16 MeV and median dose was 10 Gy (7–16 Gy). Results: Twenty-one of 23 patients were classified as high-risk. A gross total resection (GTR) was achieved in 18 patients, of whom 6 experienced disease recurrence, 2 of which included a locoregional relapse as a component of failure. Fourteen of 18 patients receiving IORT after a GTR are disease-free survivors. A second subset of 5 patients had a subtotal resection (STR), with gross residual disease remaining after surgery. All 5 patients recurred locally, and all died of their disease. IORT was extremely well-tolerated in our cohort. Surgical resection and IORT resulted in the narrowing of the abdominal aorta and an atrophic kidney in 1 patient. Conclusions: For high-risk neuroblastoma patients, IORT as the only radiotherapy to the primary, produced excellent local control after a GTR. However, IORT as the sole radiotherapy to the primary was inadequate for patients with extensive adenopathy or an STR. In this setting, we are exploring the use of IORT as a boost in conjunction with external beam radiation therapy.

Report of the first workshop on prognostic factors in large-cell lymphomas

Malignant lymphomas are a group of extremely heterogeneous diseases. This heterogeneity is seen in histological subtype as well as in clinical presentation, response to treatment, and prognosis. The natural diversity of histologic subtypes of malignant lymphomas was complicated by the number of classifications used around the world, including the Rappaport [1], Lukes-Collins [2], and Kiel [3]. However, the development of the Working Formulation [4] allowed pathologists and physicians to speak the same language. The most frequent subtype of non-Hodgkin’s lymphoma is called ‘diffuse large-cell’ lymphoma. It includes diffuse large cell and immunoblastic (groups G and H) from the Working Formulation, and is also known as diffuse histiocytic lymphoma (Rappaport), or immunoblastic and centroblastic (Kiel). In this subtype, clinical presentation can vary from localized adenopathy or localized visceral infiltration in some patients to extensive adenopathy and visceral involvement in others. Non-Hodgkin’s lymphomas are usually classified by the Ann Arbor staging system, which was primarily devised for Hodgkin’s disease [5].

Hilar and mediastinal adenopathy in sarcoidosis as detected by computed tomography

CT of the chest was performed in 25 patients with chest radiographs suspicious for hilar or mediastinal adenopathy, who subsequently proved to have sarcoidosis. In each case, CT detected more extensive adenopathy than suspected on chest radiographs. Adenopathy greater than 1.0 cm was present in the right paratracheal and pretracheal regions in all cases. Adenopathy was also frequently seen in the hilar (92%), anteroposterior window (88%), subcarinal (64%), anterior mediastinal (48%), and posterior mediastinal (16%) lymph node groups. The adenopathy occurred in multiple and varying combinations without a consistent identifiable pattern suggestive of sarcoidosis. It is concluded that while CT detects adenopathy more consistently, it does not offer a clear advantage over chest radiography in confirming a diagnosis of mediastinal and hilar involvement by sarcoidosis.

Lung and heart-lung transplantation for end-stage lung disease

Between February 1988 and December 1989, 15 combined heart-lung, 2 double lung and 5 single lung transplants were performed at our institution for end stage lung disease. The indication for heart-lung transplantation was primary lung disease with associated secondary heart failure in 11 cases, diffuse pulmonary disease with extensive adenopathy of the hilum in 2 cases and profuse and antibiotic-resistant tracheobronchial infection due to Pseudomonas in 2 cases. A double lung transplant was performed in 2 patients with hypertensive emphysema. The indication for a single lung transplantation was emphysema in 2 cases and pulmonary fibrosis in 3 cases; in this last indication, transplantation should be performed on the right side with a slight lengthening of the main bronchus to avoid the side-effects of mediastinal shift. There were 2 early deaths, 7 secondary deaths (from the 2nd to the 5th month) due to viral or bacterial infectious complications, and 1 late death in the 7th month (infection due to a syncitial virus). All 12 surviving patients have an excellent functional result; the size of the tracheal or bronchial anastomosis ranges from 85% to 100% of normal. From this experience, we conclude that specificity and severity of lung hazards are mainly related to bronchial infection, dependence on steroids and pleural adhesions. Moreover, posttransplant pulmonary oedema, mucociliary dysfunction and the differential diagnosis between rejection and infection require careful endobronchial suction and periodical sampling.