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L D Thompson

Publications and source records attributed to L D Thompson.

At least 37 records · Page 2Linked to original sources

Splenic inflammatory myofibroblastic tumor (inflammatory pseudotumor): a clinicopathologic and immunophenotypic study of 12 cases.

CONTEXT: Inflammatory pseudotumor is an uncommon and enigmatic lesion. The spindle cells found in this tumor have features of myofibroblasts. Because of the indefinite relationship of these lesions with inflammatory fibrosarcoma and their indefinite biologic behavior, inflammatory pseudotumor is currently classified as inflammatory myofibroblastic tumor (IMT). To date, only case reports or small series have been published on these tumors, which are primary in the spleen. DESIGN: In this study, we describe the clinical, morphologic, and immunophenotypic findings of 12 cases of splenic IMT and examine their relationship to Epstein-Barr virus (EBV). RESULTS: The patients included 8 women and 3 men, ranging from 19 to 77 years of age (mean, 53 years; median, 60 years). Demographic data were unavailable for 1 patient. Patients generally presented with abdominal pain (n = 5) and fever (n = 4). Associated lesions included renal cell carcinoma (n = 2), colonic adenocarcinoma (n = 1), and cholecystitis (n = 1). All tumors were composed of a bland spindle cell proliferation in association with a variable mixed inflammatory component. There were 2 growth patterns, namely, a cellular spindle cell pattern and a hypocellular fibrous pattern. An immunohistochemical panel confirmed the myofibroblastic nature of the spindle cells. The spindle cells of 2 cases were immunoreactive for EBV latent membrane protein 1, whereas 6 of 10 cases were positive for EBV-encoded RNA using in situ hybridization. Follow-up was available for 8 patients; 6 were alive with no evidence of recurrence and 2 were dead of other causes. CONCLUSION: Splenic IMTs are uncommon lesions that can be distinguished from other conditions using a combination of clinical, histologic, and immunophenotypic findings. Epstein-Barr virus may play a role in the pathogenesis of splenic IMT, and there may be an association of splenic IMT with concomitant disease or malignancy. Most splenic IMTs have an excellent long-term prognosis.

Adult↗

Renal cell carcinoma to the pancreas in surgical pathology material.

BACKGROUND: Clear cell carcinomas of the pancreas are rare and more likely represent metastatic renal cell carcinoma (RCC). METHODS: Twenty-one cases of metastatic RCC to the pancreas were retrieved from the files of the Endocrine Registry of the Armed Forces Institute of Pathology. Histologic features were reviewed, special stains and immunohistochemical studies were performed, and patient follow-up data were obtained. RESULTS: The patients included 9 women and 12 men ages 47-76 years (mean, 64.4 years). Patients experienced weight loss, abdominal pain, or a mass lesion. The tumors occurred anywhere within the pancreas. The mean size of the tumors was 4.0 cm. Histologically, the tumors were comprised of clear cells with a rich vascular network. The RCC was diagnosed before (n = 17 patients; ages up to 32.7 years) or after (n = 4 patients; ages up to 13.2 years) the pancreatic metastases were discovered. Surgery was used in all patients. Adjuvant chemotherapy was used in 4 patients. From the date of the diagnosis of pancreatic metastasis, 13 patients were dead with disseminated disease (DD) (mean, 4.5 years), and 8 patients were without disease (mean, 9.0 years). From the date of the diagnosis of primary RCC, 13 patients were DD (mean, 12.7 years), and 8 patients were without disease (mean, 24.7 years). CONCLUSIONS: Although histochemical and immunohistochemical studies may help in the distinction between patients with primary versus metastatic clear cell tumors of the pancreas, clinical confirmation should be obtained. Surgical resection of the pancreatic metastatic disease is suggested, because the patient may still have a prolonged survival.

Aged↗

Primary chondrosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 14 cases with a review of the literature.

BACKGROUND: Primary chondrosarcoma of the head and neck in the pediatric age group is rare. The literature contains several single cases and small series; however, to the authors' knowledge, there has been no previous comprehensive larger study to evaluate the clinicopathologic aspects of these tumors. METHODS: Fourteen cases of chondrosarcoma of the head and neck from patients age 18 years or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology. No secondary sarcomas (radiation-induced or arising in association with Maffucci syndrome or Ollier disease) were included. Clinical, radiographic, and histologic features were reviewed and patient follow-up obtained. RESULTS: The patients included 6 girls and 8 boys ages 3-18 years (mean, 11.8 years). Patient symptoms (nasal stuffiness or discharge, sinusitis, headaches, or a mass lesion) were related to tumor location and were present for an average of 7.2 months. No genetic abnormalities were documented. The tumors most frequently involved the maxillary sinus (n=4), followed by the mandible (n=3), nasal cavity (n=2), and neck (n=2), with 1 each of the nasopharynx, orbit, and base of the skull. The tumors ranged in size from 2.0 to 15.0 cm (mean, 3.1 cm). All tumors were invasive and malignant as determined by radiology and/or histology. The tumors were Grade 1 (n=9), Grade 2 (n=1), or Grade 3 (mesenchymal, n=2; dedifferentiated n=2). All patients were treated by surgery, followed by radiation (n=5) and/or chemotherapy (n=2). Follow-up was available for 11 patients; all were alive (at a mean of 14.8 years), with only a single patient demonstrating evidence of residual/ recurrent tumor (at 16.6 years). CONCLUSIONS: Primary head and neck chondrosarcoma in the pediatric population is typically low grade and occurs in the maxillary sinus or mandible. Despite the invasive and high grade nature of some of these tumors, there is an excellent long term prognosis for patients in this age group with tumors in these locations.

Adolescent↗

Primary thyroid teratomas: a clinicopathologic study of 30 cases.

BACKGROUND: Primary thyroid teratomas are rare thyroid gland neoplasms of germ cell derivation that display features of trilineage differentiation. METHODS: The histologic and immunophenotypic features of 30 cases of thyroid teratomas were reviewed, patient follow-up was obtained, and the results were analyzed statistically. RESULTS: The patients included 15 females and 15 males ages newborn-56 years (mean, 12.4 years). All patients presented clinically with a mass in the thyroid, ranging in size from 2.0-13 cm in greatest dimension (mean, 6.0 cm). Histologically, the tumors usually were well circumscribed, although occasionally infiltrative into the thyroid parenchyma. Derivatives of all three germ layers (ectoderm, mesoderm, and endoderm) were present in varying degrees of maturity. The tumors were divided into benign (n = 7 tumors), immature (n = 14 tumors), and malignant (n = 9 tumors) as determined by an increasing percentage of tumor volume comprised of primitive mesenchymal or neural-type tissue. All the microscopically malignant tumors occurred in the adult population. Surgical excision was performed in 28 patients, followed by adjuvant therapy in 5 patients. Follow-up was obtained in 26 patients; 8 patients had died from or with tumor (5 neonates with immature histology and 3 adults with malignant histology; mean, 0.6 years) and 18 patients were alive with no evidence of disease at a mean of 16.9 years of follow-up. CONCLUSIONS: Thyroid teratomas are rare neoplasms that can be divided into three types depending on the presence and proportion of the immature component. The outcome is dependent largely on the age of the patient, the size of the tumor at the time of initial presentation, and the presence and proportion of immaturity. Surgical excision is the treatment of choice, with adjuvant therapy reserved for the malignant cases.

Adolescent↗

Kaposi sarcoma of major salivary gland origin: A clinicopathologic series of six cases.

BACKGROUND: Kaposi sarcoma (KS), one of the defining tumors of acquired immune deficiency syndrome (AIDS), is rarely identified in the major salivary glands. To the authors' knowledge, no previous published series has evaluated the clinicopathologic aspects of this tumor in major salivary glands. METHODS: Six cases of salivary gland KS, diagnosed between 1970 and 1998, were retrieved from the files of the Oral and Maxillofacial Pathology Registry of the Armed Forces Institute of Pathology. Histologic features were reviewed and special stains, immunohistochemical studies, and in situ hybridization were performed (n = 4). Patient follow-up data were obtained. RESULTS: The patients included 6 men ages 20-73 years (average, 53.0 years). Patients presented clinically with a mass in the submandibular (n = 4) or parotid (n = 2) gland region. Symptoms were present for a mean of 13.7 months. The tumors measured 1-4 cm (average, 2.5 cm) in greatest dimension. Histologically, the tumors exhibited the usual features of KS: a spindle cell vascular proliferation arranged in fasciculated bundles, variable nuclear pleomorphism, mitotic figures, extravasated erythrocytes, and hyaline globules. Five patients were serologically positive for human immunodeficiency virus (HIV) (three homosexual males, one infected by a contaminated blood transfusion, and one with an unknown risk factor). Human herpesvirus-8 (HHV-8) was present in all cases tested (n = 4). Patients were treated with surgical excision (n = 6), followed by chemotherapy (n = 1) for the single patient with other foci of KS (rectal). Three patients died of AIDS-related infectious complications and one of congestive heart failure, whereas the remaining patients are alive with AIDS but free of salivary gland KS. CONCLUSIONS: Salivary gland enlargement is frequently identified in HIV positive or AIDS patients. Although rare, it is important to consider KS in the differential diagnosis of other AIDS-related salivary gland manifestations (infections and tumors).

AIDS-Related Opportunistic Infections↗

Infradiaphragmatic totally anomalous pulmonary venous return with two separate descending veins in association with right atrial isomerism.

We present the case of a 1-month-old infant with right atrial isomerism and complex functionally univentricular heart disease, in whom totally anomalous pulmonary venous return drained below the diaphragm via two separate vertical veins. One of the descending vertical veins drained the entire right lung and the left upper lobe, while the other drained the remainder of the left lung. Only one similar case has been described previously.

Anastomosis, Surgical↗

Retroperitoneal peripheral hemangioblastoma: a case report and review of the literature.

Central nervous system hemangioblastomas are uncommon tumors of controversial etiology that are usually found in the posterior fossa of the cranial cavity, retina, and spinal cord. Peripheral involvement is rare; only isolated case reports have been identified. We report an unusual case of hemangioblastoma involving the retroperitoneum. A 47-year-old African-American man presented with polycythemia on routine laboratory testing. Computed tomography revealed a large retroperitoneal mass near the pancreas, in a left suprarenal location, without adrenal involvement and without attachment to a nerve. Although hemangioblastoma may be associated with the von Hippel-Lindau syndrome, this patient did not have any of the stigmata of this disease. The histologic features included a highly vascular tumor with cellular areas composed of plump, pleomorphic spindled and epithelioid (stromal) cells with variable cytoplasmic lipid vacuoles and hypocellular areas with inflammatory cells and collagenous fibrils. Immunohistochemical staining showed that the tumor (stromal) cells were positive for vimentin, calponin, S-100 protein, neuron-specific enolase, and CD57 and negative for glial fibrillary acidic protein, cytokeratins, epithelial membrane antigen, CD34, HMB-45, desmin, and the actins. These morphologic and immunohistochemical findings are consistent with hemangioblastoma. To our knowledge this is the first reported case of a hemangioblastoma in this location. Based on this case we conclude that hemangioblastoma may occur in the retroperitoneum and outside of the central nervous system in a patient without von Hippel-Lindau syndrome. The immunoprofile of this case suggests that hemangioblastomas are mesenchymal neoplasms exhibiting both neural and myofibroblastic differentiation.

Hemangioblastoma↗

Surgical approach to complicated cervical aortic arch: anatomic, developmental, and surgical considerations.

BACKGROUND: Abnormalities of brachiocephalic arterial branching and arch laterality are common in patients with a cervical aortic arch. In addition, structural anomalies of the arch such as obstruction, aneurysms, and tortuosity are found in a significant number of cases. METHODS: Between 1990 and 1998, 6 patients underwent surgery for an obstructed right cervical arch. A significant obstruction was present at the transverse or distal arch in all patients, and was recurrent after previous repair in 2. In 1 patient, there was also a multi-lobed aneurysm of the aortic segment contiguous to the obstruction, and in 2 there was marked tortuosity of the arch. In all cases, the order of origin of the head and neck vessels was abnormal, and obstruction of 1 or more brachiocephalic vessels was found in 3. A vascular ring was present in all patients, with a right aortic arch and aberrant left subclavian artery in 4 patients and a double aortic arch with a dominant right cervical arch in 2. The descending aorta was circumflex (left-sided) in 3 patients. Three patients were repaired through a standard right posterolateral thoracotomy, and 3 through a median sternotomy. Patch augmentation aortoplasty was used in 2 patients, a tube graft from the ascending to descending aorta in 2, end to side anastomosis of the descending aorta to the proximal arch in 1, and direct anastomosis to reconstruct an atretic left-sided component of a double arch in 1. RESULTS: Repair was successful in all cases, with no perioperative complications. At follow-up ranging from 1 to 9 years, all patients were alive and well, with no recurrence of arch obstruction or other significant complications. Fluorescent in situ hybridization revealed microdeletion of chromosome 22q11 in 1 patient (not performed in the others). CONCLUSIONS: Structural anomalies of the arch are relatively common in patients with a cervical aortic arch. Such abnormalities may be the result of hemodynamic conditions and/or abnormal vascular tissue related either to the cervical position of the arch or its embryologic precursors. Given the highly variable anatomy of patients with a complicated cervical aortic arch, surgical considerations will vary in kind.

Adolescent↗

Amyloidosis of the larynx: a clinicopathologic study of 11 cases.

Laryngeal amyloidosis (LA) is uncommon and poorly understood, with limited long-term clinicopathologic and immunophenotypic studies in the literature. Eleven cases of LA were retrieved from the files of the Otorhinolaryngic-Head & Neck Tumor Registry from 1953 to 1990. The histology, histochemistry, immunohistochemistry, and follow-up were reviewed. All patients (three women and eight men) presented with hoarseness at an average age of 37.8 years. The lesions, polypoid or granular, measured an average of 1.6 cm and involved the true vocal cords only (n = 4), false vocal cord only (n = 1), or were transglottic (n = 6). An acellular, amorphous, eosinophilic material was present in the stroma, often accentuated around vessels and seromucous glands, which reacted positively with Congo red. A sparse lymphoplasmacytic infiltrate was present in all cases that demonstrated light chain restriction by immunohistochemistry in three cases (kappa = 2, lambda = 1). Serum and urine electrophoreses were negative in all patients. Treatment was limited to surgical excision, including a single laryngectomy. Six patients manifested either recurrent and/or multifocal/systemic disease: two patients with light chain restriction were dead with recurrent disease (mean, 11.1 years); two patients were dead with no evidence of disease (mean, 31.7 years); and two patients were alive, one with light chain restriction and recurrent and multifocal disease (41.6 years) and one with no evidence of disease after a single recurrence (43.4 years). The remaining five patients were either alive or had died with no evidence of disease an average of 32.4 years after diagnosis. No patient developed multiple myeloma or an overt B-cell lymphoma. LA is an uncommon indolent lesion that may be associated with multifocal disease (local or systemic). The presence of an associated monoclonal lymphoplasmacytic infiltrate and recurrent/multifocal disease in the respiratory or gastrointestinal tract of a few cases and the lack of development of a systemic plasma cell dyscrasia or overt systemic B-cell malignancy suggest that some LA may be the result of an immunocyte dyscrasia or tumor of mucosa-associated lymphoid tissue.

Adult↗

Splenic angiosarcoma: a clinicopathologic and immunophenotypic study of 28 cases.

Primary angiosarcoma of the spleen is a rare neoplasm that has not been well characterized. We describe the clinical, morphologic, and immunophenotypic findings of 28 cases of primary splenic angiosarcoma, including one case that shares features of lymphangioma/lymphangiosarcoma. The patients included 16 men and 12 women, aged 29 to 85 years, with a mean of 59 years and median of 63 years. The majority of patients (75%) complained of abdominal pain, and 25% presented with splenic rupture. The most common physical finding was splenomegaly (71%). Seventeen of 21 patients were reported to have anemia. Macroscopic examination showed splenomegaly in 85% cases. Sectioning revealed discrete lesions in 88% of cases, ranging from well-circumscribed firm nodules to poorly delineated foci of necrosis and hemorrhage associated with cystic spaces. Microscopically, the tumors were heterogenous; however, all cases demonstrated at least a focal vasoformative component lined by atypical endothelial cells. Solid sarcomatous, papillary, and epithelioid growth patterns were observed. The solid sarcomatous component resembled fibrosarcoma in two cases and malignant fibroushistiocytoma in one case. Hemorrhage, necrosis, hemosiderin, extramedullary hematopoiesis, and intracytoplasmic hyaline globules were frequently identified. A panel of immunohistochemical studies revealed that the majority of tumors were immunoreactive for at least two markers of vascular differentiation (CD34, FVIIIRAg, VEGFR3, and CD31) and at least one marker of histiocytic differentiation (CD68 and/or lysozyme). Metastases developed in 100% of patients during the course of their disease. Twenty-six patients died of disease despite aggressive therapy, whereas only two patients are alive at last follow-up, one with disease at 8 years and the other without disease at 10 years. In conclusion, primary splenic angiosarcoma is an extremely aggressive neoplasm that is almost universally fatal. The majority of splenic angiosarcomas coexpress histiocytic and endothelial markers by immunohistochemical analysis, which suggest that some tumors may originate from splenic lining cells.

Adult↗

Tonsillar lymphangiomatous polyps: a clinicopathologic series of 26 cases.

BACKGROUND: Lymphangiomatous polyps are uncommon benign tumors of the tonsils. METHODS: Twenty-six cases of lymphangiomatous polyps diagnosed between 1980 and 1999 were retrieved from the files of the Otorhinolaryngic-Head and Neck Tumor Registry of the Armed Forces Institute of Pathology. Hematoxylin and eosin-stained slides were reviewed to characterize the histologic features of these tumors. Immunohistochemical stains were performed on 15 cases. Clinical follow-up data were obtained. RESULTS: The patients included 13 males and 13 females, ages 3 to 63 years (mean, 25.2 years). Patients experienced dysphagia, sore throat, and the sensation of a mass in the throat. Symptoms were present from a few weeks to years. The tonsillar masses were unilateral in all cases. Clinically, the lesions were frequently mistaken for a neoplasm (n = 18 patients). Grossly, all of the lesions were polypoid and measured 0.5 to 3.8 cm (mean, 1.6 cm). Histologically, the polyps were covered by squamous epithelium showing variable epithelial hyperplasia, dyskeratosis, and lymphocytic epitheliotropism. The masses showed a characteristic submucosal proliferation of small to medium-sized, endothelial-lined, lymph-vascular channels lacking features of malignancy. Collagen, smooth muscle, and adipose tissue were present in the stroma. Intravascular proteinaceous fluid and lymphocytes were noted. Immunohistochemical findings confirmed the endothelial origin of the vascular proliferation and a mixed lymphoid population. The differential diagnosis included fibroepithelial polyp, lymphangioma, juvenile angiofibroma, and squamous papilloma. In all patients with follow-up, complete surgical excision was curative (mean follow-up, 5.4 years; range, 1 mo to 14 years). CONCLUSIONS: We detail the clinical and pathologic features of tonsillar lymphangiomatous polyps. These tumors are uncommon and may clinically be mistaken for a malignant neoplasm. The characteristic histologic features should allow for its correct diagnosis and differentiation from similar appearing tonsillar lesions.

Adolescent↗

Sinonasal mucosal malignant melanoma: report of an unusual case mimicking schwannoma.

Primary mucosal melanoma of the sinonasal tract is a rare malignancy that has a more aggressive clinical course than its cutaneous counterpart. The histology of these lesions varies, with differing degrees of melanin production and an epithelioid or spindle-cell growth pattern. Cutaneous melanocytic lesions may differentiate in accordance with their neural crest derivation and express morphology similar to nerve sheath tumors. We believe the following case study reports the first instance of a mucosal melanoma with a Schwannian pattern of growth, arising from the nasal cavity of a 26-year-old man.

Adult↗

Malignant lymphoma of the thyroid gland: a clinicopathologic study of 108 cases.

We report a retrospective clinicopathologic study of 108 primary thyroid gland lymphomas (PTLs), classified using the REAL and proposed WHO classification schemes. The patients included 79 women and 29 men, with an average age of 64.3 years. All patients presented with a thyroid mass. The PTLs were classified as marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) or MZBL (n = 30), diffuse large B-cell lymphoma (DLBCL) with MZBL (n = 36), DLBCL without MZBL (n = 41), and follicle center lymphoma (FCL; n = 1). Excluding the FCL, features of lymphomas of MALT-type were identified in all groups, despite a follicular architecture in 23% of cases. Lymphocytic thyroiditis (LT) was identified in 94%. Ninety-one percent of patients presented with stage IE or IIE disease, whereas 69% had perithyroidal soft tissue infiltration. All patients were treated with surgical excision followed by adjuvant therapy (76%): chemotherapy (15%), radiation (19%), or a combination of radiation and chemotherapy (42%). Disease-specific survival was 82% at last follow up (mean, 82.8 mos) and 79% at 5 years. Statistically, stages greater than IE, presence of DLBCL, rapid clinical growth, abundant apoptosis, presence of vascular invasion, high mitotic rate, and infiltration of the perithyroidal soft tissue were significantly associated with death with disease. No patients with MZBL or stage IE disease died with disease. In summary, PTLs typically occur in middle- to older-aged individuals as a thyroid mass, with a predilection for females. Despite their histologic heterogeneity and frequent simulation of other lymphoma subtypes, virtually all PTLs are lymphomas of MALT-type arising in the setting of LT. Mixed DLBCL and MZBL are common. Overall, PTLs have a favorable outcome with appropriate therapy, but prognosis depends on both clinical stage and histology. MZBL and stage IE tumors have an excellent prognosis, whereas tumors with a large cell component or DLBCL or stage greater than IE have the greatest potential for a poor outcome.

Adult↗

Extracranial sinonasal tract meningiomas: a clinicopathologic study of 30 cases with a review of the literature.

Extracranial meningiomas of the sinonasal tract are rare tumors. These tumors are frequently misclassified, resulting in inappropriate clinical management. To date, there has been no comprehensive study to evaluate the clinicopathologic aspects of meningioma in these anatomic sites. Thirty cases of sinonasal tract meningiomas diagnosed between 1970 and 1992 were retrieved from the files of the Otorhinolaryngic Registry of the AFIP. Histologic features were reviewed, immunohistochemical studies were performed, patient follow up was obtained, and the results were statistically analyzed. The patients included 15 females and 15 males, aged 13 to 88 years (mean, 47.6 yrs). Patients presented clinically with a mass, epistaxis, sinusitis, pain, visual changes, or nasal obstruction, dependent on the anatomic site of involvement. Symptoms were present for an average of 31.1 months. The tumors affected the nasal cavity (n = 14), nasopharynx (n = 3), frontal sinus (n = 2), sphenoid sinus (n = 2). or a combination of the nasal cavity and ethmoid, frontal, sphenoid, and/or maxillary sinuses (n = 9). The tumors ranged in size from 1.0 to 8.0 cm in greatest dimension (mean, 3.5 cm). Radiographic studies demonstrated a central nervous system connection in six cases. The tumors often eroded the bones of the sinuses (n = 18) and involved the surrounding soft tissues, the orbit, and occasionally the base of the skull. Histologically, the tumors demonstrated features similar to intracranial meningiomas. The majority were of the meningothelial type (n = 23), although there were three atypical meningiomas. Immunohistochemical studies confirmed the diagnosis of meningioma with positive reactions for epithelial membrane antigen (EMA) and vimentin (all tested). The differential diagnosis includes paraganglioma, carcinoma, melanoma, psammomatoid ossifying fibroma, and angiofibroma. Surgical excision was used in all patients. Three patients died with recurrent disease (mean, 1.2 yrs), one was alive with recurrent disease (25.6 years), and the remaining 24 patients were alive or had died of unrelated causes (mean, 13.9 yrs) at the time of last follow up (two patients were lost to follow up). Extracranial sinonasal tract meningiomas are rare tumors which need to be considered in the differential diagnosis of sinonasal tumors. A whorled growth pattern and psammoma bodies, combined with positive EMA and vimentin immunohistochemical reactions, can confirm the diagnosis of meningioma. The overall prognosis is good, without a difference in outcome between benign and atypical meningiomas.

Adolescent↗

A clinicopathologic series of 22 cases of tonsillar granulomas.

OBJECTIVES: Tonsils are uncommonly affected by granulomatous inflammation, often with an obscure cause. This study attempts to elucidate the nature of tonsillar granulomatous inflammation. DESIGN: Retrospective clinicopathologic review. METHODS: Twenty-two cases of tonsillar granulomas diagnosed between 1940 and 1999 were retrieved from the files of the Armed Forces Institute of Pathology. H&E slides and a series of histochemical stains were reviewed, and patient follow-up was obtained. RESULTS: There were 11 males and 11 females, aged 7 to 64 years (mean, 29.9 y). Most of the cases presented bilaterally (n = 19) with sore throat, dysphagia, and/or nasal obstruction. The clinical differential included chronic tonsillitis, tuberculosis, nonspecific infection, sarcoidosis, and a neoplasm. Histologically, the granulomas were focal and scattered, or diffuse, identified in the interfollicular zones (n = 16) and/or the germinal centers (n = 13), and occasionally associated with necrosis (n = 6). Based on histochemical and clinical follow-up information, the etiology of the granulomas included sarcoidosis (n = 8), tuberculosis (n = 3), Hodgkin's lymphoma (n = 2), toxoplasmosis (n = 1), squamous cell carcinoma (n = 1), and no specific known cause (n = 7). Twelve patients were either alive at last follow-up or had died with no evidence of disease (mean, 12.4 y), and 9 were either alive at last follow-up or had died with disease (mean, 24.9 y). One patient was alive with unknown disease status (lost to follow-up after 13.3 y). CONCLUSIONS: Although a cause for tonsillar granulomas is frequently identified, a number may not develop an identifiable etiology, with the granulomas probably representing an exaggerated immune response to chronic tonsillitis. However, a careful work-up must be conducted to exclude specific causes and avoid clinical mismanagement.

Adolescent↗

A clinicopathological study of 15 patients with neuroglial heterotopias and encephaloceles of the middle ear and mastoid region.

OBJECTIVES/HYPOTHESIS: Heterotopic masses of neuroglial tissue involving non-midline structures, specifically, the middle ear region, are exceptional. The pathogenesis of these lesions and, in particular, their relation to encephaloceles, is uncertain. STUDY DESIGN AND METHODS: H&E-stained sections from 15 lesions diagnosed as neuroglial heterotopias or encephaloceles involving the middle ear region were reviewed. Radiographic or operative evidence of a central nervous system (CNS) relation and clinical factors possibly related to pathogenesis were analyzed. RESULTS: All 15 lesions (from six men and nine women; mean age, 49 y; range, 16-67 y), regardless of their relation to the CNS, were composed of varying proportions of neurons and glia with associated chronic inflammatory cells and reactive gliosis. No significant ependymal or choroid plexus component was present. Operative findings revealed that two lesions had definite CNS connections and two were unrelated to the CNS; this relation could not be determined in the remaining cases. Seven of 10 patients for whom clinical information was available had a history of chronic otitis media or mastoiditis or both; four of these seven patients also had a history of previous trauma or surgery. Three patients, including both patients whose lesions had no demonstrable CNS attachment, had no predisposing factors. CONCLUSIONS: Most neuroglial heterotopias of the middle ear are probably acquired encephaloceles. These lesions occur in older patients than do their midline counterparts. Determination of the relation of these lesions to adjacent CNS structures must be done radiographically or using operative findings, because histology alone cannot be reliably used to render an accurate diagnosis.

Adolescent↗

Extracardiac conduit variation of the Fontan procedure.

The ECF operation is designed to improve postoperative outcome by enhancing factors that are critical in optimal functioning of the Fontan circulation, including preservation of ventricular and pulmonary vascular function, avoidance of dysrhythmias, and prevention of stasis and flow turbulence in the Fontan circuit. Preoperative strategies include an early bidirectional Glenn procedure, and avoiding ancillary intracardiac procedures at the time of the Fontan by performing them at the time of the Glenn operation. Operative strategies include minimizing the duration of CPB by performing the conduit to pulmonary artery anastomosis off bypass, using partial instead of full CPB by cannulating the IVC alone, avoiding hypothermia, avoiding cross-clamping of the aorta, avoiding atrial incisions and suture lines, using a tubular conduit to construct the Fontan pathway, making a large conduit to pulmonary artery anastomosis, incorporating the conduit into aggressive pulmonary arterioplasties, and offsetting of the superior and inferior cavopulmonary anastomoses.

Arrhythmias, Cardiac↗