Fungal Prostatitis Due to Endemic Mycoses and Cryptococcus: A Multicenter Case Series

Other Anatomic Sites, Prostate
Epstein DJ, Thompson LDR, Saleem A, Kao CS, Epstein JI.
Prostate. 2020 Jun 22. doi: 10.1002/pros.24034. Online ahead of print.
BACKGROUND: Fungal prostatitis is exceedingly rare with mostly case reports.
METHODS: Electronic medical records at three medical centers were searched for cases of fungal prostatitis due to endemic mycoses and Cryptococcus over the preceding 10 years.
RESULTS: Seven cases were identified from 105 600 prostate biopsies within the Southern California Permanente Medical Group for an incidence of 0.0066%. An additional eight cases were identified from two other health care systems. Excluding four patients without available clinical data, 11 patients were reviewed, most of whom underwent biopsy due to elevated prostate-specific antigen. Four were asymptomatic and the remainder had nonspecific signs or symptoms. All biopsies revealed granulomatous inflammation and fungal organisms. Seven patients had coccidioidomycosis, three patients had cryptococcosis (confirmed in two cases and suspected by organism morphology in the other), and one patient had likely histoplasmosis based on organism morphology. Prolonged antifungal treatment was standard; outcomes were favorable.
CONCLUSION: Fungal prostatitis due to endemic mycoses and Cryptococcus is uncommon and associated with favorable outcomes but generally involves prolonged therapy.
PubMed ID: 32572997
Article Size: 1.6 MB

Hailey-Hailey disease.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2016 Sep;95(9):370.
FIRST PARAGRAPH: Hailey-Hailey disease is a genodermatosis characterized by incomplete penetrance of an autosomal dominant disorder with defects in the ATP2C1 gene. Patients present with well-demarcated, erythematous plaques that often show crusting. The plaques may be vegetative, commonly associated with a distinct malodor. There may be associated burning or itching. While intertriginous areas are most commonly affected, the neck is also frequently involved. There is a natural remission and exacerbation cycle, with heat, moisture, and trauma the most common exacerbating factors. The lesions may be managed by corticosteroids or antibiotics, along with retinoids for severe disease.
PubMed ID: 27657314
Article Size: <1 MB

Myeloid sarcoma of the breast soft tissue following allogeneic bone marrow transplantation

Other Anatomic Sites, Soft Tissue
Chou JL, Thompson LD.
J Case Rep Images Oncology 2016;2:71–73.
FIRST PARAGRAPH: A 47-year-old female presented with high-risk myelodysplastic syndrome (MDS) without detectable cytogenetic abnormality from the bone marrow specimen. Initially, she received azacytidine while awaiting bone marrow transplantation (BMT). After successful allogeneic BMT from a mismatched related donor, she received post-transplant care including management of transplant-related toxicity and skin manifestations of graft versus host disease (GVHD) in a designated BMT center. Subsequent bone marrow showed no evidence of MDS, and she is currently free from GVHD.
Article Size: <1 MB

Primary extracranial meningiomas: An analysis of 146 cases.

Other Anatomic Sites, Soft Tissue
Rushing, EJ, Bouffard JP, McCall S, Olsen C, Mena H, Sandberg GD, Thompson LDR.
Head Neck Pathol. 2010 Mar;4(1):37-43.
Primary extracranial meningiomas are rare neoplasms, frequently misdiagnosed, resulting in inappropriate clinical management. To date, a large clinicopathologic study has not been reported. One hundred and forty-six cases diagnosed between 1970 and 1999 were retrieved from the files of the Armed Forces Institute of Pathology. Histologic features were reviewed, immunohistochemistry analysis was performed (n = 85), and patient follow-up was obtained (n = 110). The patients included 74 (50.7%) females and 72 (49.3%) males. Tumors of the skin were much more common in males than females (1.7:1). There was an overall mean age at presentation of 42.4 years, with a range of 0.3–88 years. The overall mean age at presentation was significantly younger for skin primaries (36.2 years) than for ear (50.1 years) and nasal cavity (47.1 years) primaries. Symptoms were in general non-specific and reflected the anatomic site of involvement, affecting the following areas in order of frequency: scalp skin (40.4%), ear and temporal bone (26%), and sinonasal tract (24%). The tumors ranged in size from 0.5 up to 8 cm, with a mean size of 2.3 cm. Histologically, the majority of tumors were meningothelial (77.4%), followed by atypical (7.5%), psammomatous (4.1%) and anaplastic (2.7%). Psammoma bodies were present in 45 tumors (30.8%), and bone invasion in 31 (21.2%) of tumors. The vast majority were WHO Grade I tumors (87.7%), followed by Grade II (9.6%) and Grade III (2.7%) tumors. Immunohistochemically, the tumor cells labeled for EMA (76%; 61/80), S-100 protein (19%; 15/78), CK 7 (22%; 12/55), and while there was ki-67 labeling in 27% (21/78), 3% of cells were positive. The differential diagnosis included a number of mesenchymal and epithelial tumors (paraganglioma, schwannoma, carcinoma, melanoma, neuroendocrine adenoma of the middle ear), depending on the anatomic site of involvement. Treatment and follow-up was available in 110 patients: Biopsy, local excision, or wide excision was employed. Follow-up time ranged from 1 month to 32 years, with an average of 14.5 years. Recurrences were noted in 26 (23.6%) patients, who were further managed by additional surgery. At last follow-up, recurrent disease was persistent in 15 patients (mean, 7.7 years): 13 patients were dead (died with disease) and two were alive; the remaining patients were disease free (alive 60, mean 19.0 years, dead 35, mean 9.6 years). There is no statistically significant difference in 5-year survival rates by site: ear and temporal bone: 83.3%; nasal cavity: 81.8%; scalp skin: 78.5%; other sites: 65.5% (P = 0.155). Meningiomas can present in a wide variety of sites, especially within the head and neck region. They behave as slow-growing neoplasms with a good prognosis, with longest survival associated with younger age, and complete resection. Awareness of this diagnosis in an unexpected location will help to avoid potential difficulties associated with the diagnosis and management of these tumors.
PubMed ID: 19644540
Article Size: <1 MB
 

Spindle-cell lipoma.

Other Anatomic Sites, Soft Tissue
Thompson LD.
Ear Nose Throat J. 2009 Jul;88(7):992-3.
FIRST PARAGRAPH: Histologically, spindle-cell lipoma is a distinctive type of lipoma on a continuum with pleomorphic lipoma. It accounts for approximately 1.5% of all adipose tissue neoplasms. Men are affected significantly more commonly than women (9:1) at a mean age in the sixth decade. The vast majority of tumors are located in the subcutaneous tissue of the posterior neck, upper back, and shoulders. Patients present with a painless, mobile, subcutaneous mass. In rare cases, these tumors develop in other head and neck mucosal sites, such as the buccal fat pad. Spindle-cell lipomas grow as large as 13 cm (mean: 3.5). Grossly, they resemble ordinary lipomas, although they may be somewhat firmer, especially if the spindle-cell component predominates.
PubMed ID: 19623524
Article Size: <1 MB

Skin basal cell carcinoma.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2010 Sep;89(9):418-20.
FIRST PARAGRAPH: Basal cell carcinoma (BCC) is a low-grade malignancy of basal keratinocytes, the cells responsible for epidermis formation. Melanocytes are seen between the keratinocytes, but they are not responsible for this tumor type. The etiology is multifactorial, related to ultraviolet sun exposure, radiation, and immunosuppression, among other factors. This is one of the most common cancers in humans. The tumors will typically present in older adults, although young adults can also develop this tumor. There is a slight male predilection, but this may be due to differences in sun exposure rather than gender variance. Caucasians and light-skinned people have a higher incidence than dark-skinned people.
PubMed ID: 20859866
Article Size: <1 MB

Pilomatricoma.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2012 Jan;91(1):18-20.
FIRST PARAGRAPH: Pilomatricoma, also referred to as pilomatrixoma and calcifying epithelioma of Malherbe, is a benign dermal-subcutaneous tumor derived from the matrix of the hair follicle. Its development is associated with a known mutation in the CTNNB1 gene, the gene that encodes for beta-catenin. Pilomatricomas are relatively common tumors. They usually arise during the first 2 decades of life, and they have no predilection for either sex.
PubMed ID: 22278863
Article Size: <1 MB

Orbital infantile myofibroma: a case report and clinicopathologic review of 24 cases from the literature.

Other Anatomic Sites, Soft Tissue
Mynatt CJ, Feldman KA, Thompson LD.
Head Neck Pathol. 2011 Sep;5(3):205-15.
Isolated orbital infantile myofibroma are rare tumors in the head and neck. The mass-like clinical presentation and variable histologic features result in frequent misdiagnosis and potentially inappropriate clinical management. There are only a few reported cases in the English literature. Twenty-four patients with orbital infantile myofibroma or myofibromatosis were compiled from the English literature (Medline 1960–2011) and integrated with this case report. The patients included 14 males and 10 females, aged newborn to 10 years (mean, 34.8 months), who presented with a painless mass in the infra- or supraorbital regions, usually increasing in size andassociated with exophthalmos (n = 5). Females were on average older than their male counterparts (38.9 vs. 31.9 months, respectively; P = 0.71). The tumors were twice as frequent on the left (n = 16) than right (n = 8). Patients experienced symptoms for an average of 2.7 months before clinical presentation. The tumors involved the bone (n = 17) or the soft tissues (n = 7) of the orbit, with extension into the nasal or oral cavity (n = 3). The mean size was 3.0 cm, with a statistically significant difference between males and females (mean: 3.9 vs. 1.82; P = 0.0047), but without any differences based on age at presentation (P = 0.25), duration of symptoms (P = 0.66), or bone or soft tissue involvement (P = 0.51). Grossly, all tumors were wellcircumscribed, firm to rubbery, homogenous, and white– grey. Histologically, the tumors were biphasic, showing whorled and nodular areas of fusiform cells with extracellular collagen, mixed with a population of small, primitiveappearing, darkly staining cells. Necrosis was not present, but mitoses could be seen. Tumors with immunohistochemistry performed showed strong and diffuse smooth muscle actin and vimentin immunoreactivity, but were negative with muscle specific actin, desmin, MYOD1, myogenin, S100 protein, GFAP, keratin, CD31, 34, Factor VIIIR-Ag, and CD45RB. The principle histologic differential diagnosis includes juvenile hyaline fibromatosis, fibrous hamartoma of infancy, fibromatosis coli, leiomyoma, infantile hemangiopericytoma, infantile fibrosarcoma, Ewing sarcoma/primitive neuroectodermal tumor, and lymphoma. All patients were managed with surgery. Recurrences developed in two patients at 4 and 6 months, respectively. Follow-up data was available on all but two patients (n = 22). These patients were either alive without evidence of disease (n = 18), alive but with disease (n = 3), or had died unrelated to this disease (i.e., neuroblastoma, n = 1). Orbital infantile myofibroma is a rare tumor, presenting in infancy as an enlarging mass of the orbit, with characteristic histomorphologic and immunophenotypic features. Orbital disease is usually isolated rather than part of systemic disease, and shows an excellent long-term prognosis, making appropriate separation from other conditions important.
PubMed ID: 21512784
Article Size: 2.5 MB
 

Intradural, extramedullary spinal cord granular cell tumor: a case report and clinicopathologic review of the literature.

Neuro (brain), Other Anatomic Sites
Weinstein BJ, Arora T, Thompson LD.
Neuropathology. 2010 Dec;30(6):621-6.
Granular cell tumor (GCT) of the spine is uncommon, with intradural extramedullary location being exceptionally rare. The non-specific clinical presentation and variable histologic patterns can make recognition of this tumor challenging. Two previous reports of GCT of the spine were reviewed (Medline 1960-2009) and analyzed with respect to this case report. The patients included two women and one man (mean age, 28.7 years). Patients presented with 3 to 4 months of lower back pain and/or lower extremity radiculopathy. The lesions appeared radiographically to be intradural and extramedullary or intramedullary. The tumors were found at T10 or L1-L2 space. Radiographically, all tumors enhanced homogenously on T1 post-gadolinium imaging with a mean tumor size of approximately 1.6 cm. Histologically, the tumors were composed of large, polygonal granular cells. The abundant cytoplasm was fine or coarsely granular, surrounding small, pale-staining nuclei, which were eccentrically located in the cell. The tumor cells were periodic acid Schiff positive, diastase resistant, and were positive with S-100 protein, CD68, inhibin, and neuron-specific enolase immunohistochemistry. The clinical and histologic differential diagnosis includes schwannoma, neurofibroma, meningioma, astrocytoma, melanocytoma, and metastatic tumors. Patients were managed with excision. One patient had symptomatic and radiographic local recurrence that was subsequently treated with radiation, resulting in stabilization of disease and symptoms. Intradural GCTs of the spine are rare and radiographically indistinguishable from tumors that more commonly arise in this location. Histologic recognition of this rare tumor is important because the subsequent clinical course of the disease differs from other similar lesions.
PubMed ID: 20113407
Article Size: 1 MB
 

Merkel cell carcinoma.

Other Anatomic Sites, Skin
Shiffer JD, Thompson LD.
Ear Nose Throat J. 2014 Mar;93(3):100-2.
FIRST PARAGRAPH: Merkel cell carcinoma is a rare and highly aggressive cutaneous neuroendocrine carcinoma that, in most cases, is caused by Merkel cell polyomavirus (approximately 80% of cases). The tumor usually arises on sun-exposed skin of elderly patients, particularly on the head and neck and extremities. There is a slight female preponderance and an increased risk in individuals who are immunosuppressed. Clinically, the tumors are often indistinguishable from other skin cancers and typically present as a firm, painless, rapidly growing nodule. They may be flesh-colored, red, or blue tumors that typically vary in size from 0.5 cm to more than 5 cm (average, 2 cm). When they have a reddish nodular appearance, they may be mistaken for angiosarcoma or granulation tissue.
PubMed ID: 24652557
Article Size: <1 MB

Osteomyelitis.

Bone, Other Anatomic Sites
Gannon FH, Thompson LD.
Ear Nose Throat J. 2005 Nov;84(11):694.
FIRST PARAGRAPH: The proper treatment and clinical management of osteomyelitis (bone infection) depend on a successful correlation of its clinical features with radiologic and pathologic findings. Diagnostic difficulties may arise, and the final arbiter is intraoperative culture. The importance of intraoperative culture obtained in a ‘sterile’ environment cannot be overemphasized.
PubMed ID: 16381128
Article Size: <1 MB

Seborrheic keratosis.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2006 Feb;85(2):79.
FIRST PARAGRAPH: Seborrheic keratosis is a benign proliferation of the epidermis. Many names are applied to this lesion (senile wart, melanoacanthoma), but seborrheic keratosis is the most widely accepted. Considered one of the most common skin lesions, it usually occurs in older patients; there is no predilection for either sex. The lesions appear “stuck on” the skin, usually on sun-exposed skin. They can be tan, brown, or black. Frequent irritation or friction may cause a papule/nodule to become red and scaly and result in a clinical misdiagnosis. Many variants of seborrheic keratosis are recognized clinically and histologically, but they have no management implications.
PubMed ID: 16579189
Article Size: <1 MB

Melanoma.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2007 Sep;86(9):542, 545
FIRST PARAGRAPH: Melanoma is a malignancy of melanocytes that show a series of molecular events that result in the melanocytes going through a stepwise progression from dysplasia to invasion to metastasis. Melanomas account for approximately 4.4% of all malignancies. Approximately 62,000 new cases of melanoma are reported annually in the United States, and they are responsible for about 7,900 deaths. The incidence of melanoma worldwide has been increasing steadily.
PubMed ID: 17970142
Article Size: <1 MB

Traumatic fracture callus

Bone, Other Anatomic Sites
Gannon FH, Thompson L.
Ear Nose Throat J. 2007 Apr;86(4):200
FIRST PARAGRAPH: Bones of the craniofacial region are frequently broken, traumatically or iatrogenically. Whereas traumatic fractures can be readily identified clinically and radiologically, they can represent a diagnostic challenge histologically. A short discussion about the histologic evolution of traumatic fractures will help a pathologist know what to expect histologically, based on the time frames of development. The repair process follows a predictable histologic evolution of five distinct phases: culatory, cellular, vascular, metabolic, and mechanical.
PubMed ID: 17500387
Article Size: <1 MB

Skin keloid.

Other Anatomic Sites, Skin
Thompson LD.
Ear Nose Throat J. 2004 Aug;83(8):519.
FIRST PARAGRAPH: A keloid is a form of excessive scar formation that occurs in response to tissue injury. Keloids arise as a result of abnormal wound healing where there is a disruption of the balance between collagen formation and degradation with a shift toward excess synthesis. Even with injuries that are seemingly minor, the resultant scar formation is disproportionately excessive. The etiology is unknown.
PubMed ID: 15487627
Article Size: <1 MB

Myeloid sarcoma.

Hematopathology, Other Anatomic Sites
Pantanowitz L, Thompson L.
Ear Nose Throat J. 2005 Aug;84(8):470-1.
FIRST PARAGRAPH: Myeloid sarcoma (MS) is an extramedullary myeloid tumor (granulocytic sarcoma) that can occur in one of three clinical settings: (1) in patients who have a history of acute myeloid leukemia (AML), during active disease or a recurrence; (2) in patients with chronic myeloproliferative disorder or myelodysplastic syndromes, who are at increased risk of blast transformation or acute leukemia; or (3) in patients with no history of hematologic disease, although it commonly predates the development of leukemia, often within 1 year.
PubMed ID: 16220848
Article Size: <1 MB

Molluscum contagiosum.

Other Anatomic Sites, Skin
Nelson BL, Thompson LD.
Ear Nose Throat J. 2003 Aug;82(8):560.
FIRST PARAGRAPH: Molluscum contagiosum is a virus-induced epithelial hyperplasia produced by a DNA poxvirus. This common disease can be found on the skin and mucosal surfaces. It is acquired by direct contact with an infected individual or, less commonly, by contact with a fomite (e.g., clothing, towels, and toys); autoinoculation is common. Molluscum contagiosum is usually seen in children and young adults; immunocompromised persons are especially vulnerable to infection. Lesions predominantly arise on the skin of the face, neck, eyelids, trunk, and genitalia, as well as on the mucous membranes of these areas (as applicable). The incubation period averages between 2 and 7 weeks, although it can be much longer.
PubMed ID: 14503090
Article Size: <1 MB

Malignant transformation of a dysembryoplastic neuroepithelial tumor after radiation and chemotherapy.

Neuro (brain), Other Anatomic Sites
Rushing EJ, Thompson LD, Mena H.
Ann Diagn Pathol. 2003 Aug;7(4):240-4.
We describe a case of anaplastic astrocytoma in a 14-year-old boy arising at the site of a dysembryoplastic neuroepithelial tumor (DNT) 3 years after combined radiation and chemotherapy. The subtotally excised superficial right temporoparietal tumor was originally diagnosed as mixed oligoastrocytoma in 1974; the patient was treated with radiation therapy postoperatively. One year later he underwent a craniotomy to remove cyst fluid and no change was reported in the size of the residual tumor. Postoperatively, he received a 6-week course of chemotherapy (lovustine, CCNU). He remained clinically and radiographically stable until 3 years later, when seizure activity returned and imaging studies were consistent with tumor recurrence. He was lost to follow-up until 1986, when records showed that he had died. Review of the initial biopsy showed cortical fragments containing abundant calcifications and multinodular structures typical of the complex form of DNT, in addition to specific glioneuronal elements. The Ki-67 labeling index ranged from 0.1% to 3% focally. The specimen from the third surgery showed an anaplastic astrocytoma (Ki-67 up to 12%) and morphologic features characteristic of radiation effect. This is the first documented case of malignant transformation of DNT following radiation and adjuvant chemotherapy. The implications of malignant transformation in subtotally excised complex DNTs and the intriguing issue of the contribution of radiation/chemotherapy are discussed.
PubMed ID: 12913847
Article Size: <1 MB
 

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

Neuro (brain), Other Anatomic Sites
Fanburg-Smith JC, Gyure KA, Michal M, Katz D, Thompson LD.
Ann Diagn Pathol. 2000 Apr;4(2):81-7.
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.
PubMed ID: 10760321
Article Size: <1 MB
 

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

Bone, Other Anatomic Sites
Gadwal SR, Fanburg-Smith JC, Gannon FH, Thompson LD.
Cancer. 2000 May 1;88(9):2181-8.
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.
PubMed ID: 10813732
Article Size: <1 MB
 

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

Hematopathology, Other Anatomic Sites
Neuhauser TS, Derringer GA, Thompson LD, Fanburg-Smith JC, Aguilera NS, Andriko J, Chu WS, Abbondanzo SL.
Arch Pathol Lab Med. 2001 Mar;125(3):379-85.
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.
PubMed ID: 11231487
Article Size: <1 MB
 

Mast cell involvement in fibrodysplasia ossificans progressiva.

Bone, Other Anatomic Sites
Gannon FH, Glaser D, Caron R, Thompson LD, Shore EM, Kaplan FS.
Hum Pathol. 2001 Aug;32(8):842-8.
Fibrodysplasia ossificans progressiva (FOP) is a catastrophic genetic disorder of progressive heterotopic ossification associated with dysregulated production of bone morphogenetic protein 4 (BMP4), a potent osteogenic morphogen. Postnatal heterotopic ossification in FOP is often heralded by hectic episodes of severe post-traumatic connective tissue swelling and intramuscular edema, followed by an intense and highly angiogenic fibroproliferative mass. The abrupt appearance, intense size, and rapid intrafascial spread of the edematous preosseous fibroproliferative lesions implicate a dysregulated wound response mechanism and suggest that cells and mediators involved in inflammation and tissue repair may be conscripted in the growth and progression of FOP lesions. The central and coordinate role of inflammatory mast cells and their mediators in tissue edema, wound repair, fibrogenesis, angiogenesis, and tumor invasion prompted us to investigate the potential involvement of mast cells in the pathology of FOP lesions. We show that inflammatory mast cells are present at every stage of the development of FOP lesions and are most pronounced at the highly vascular fibroproliferative stage. Mast cell density at the periphery of FOP lesional tissue is 40- to 150-fold greater than in normal control skeletal muscle or in uninvolved skeletal muscle from FOP patients and 10- to 40-fold greater than in any other inflammatory myopathy examined. These findings document mobilization and activation of inflammatory mast cells in the pathology of FOP lesions and provide a novel and previously unrecognized target for pharmacologic intervention in this extremely disabling disease.
PubMed ID: 11521229
Article Size: 2 MB
 

Large-loop excision of the transformation zone: effect on the pathologic interpretation of resection margins.

Cervix, Other Anatomic Sites
Montz FJ, Holschneider CH, Thompson LD.
Obstet Gynecol. 1993 Jun;81(6):976-82.
OBJECTIVE: To determine the effect of large-loop excision of the transformation zone (LLETZ) on the ability to interpret adequately the pathologic specimen and surgical margins.
METHODS: Fifty consecutive LLETZ specimens were used for repeat histopathologic assessment with emphasis on the interpretability of the surgical specimen and margin. All reevaluations were performed by a single pathologist. Complete lesion evaluability was defined as satisfactory accuracy of the histologic diagnosis and the ability to evaluate thoroughly all surgical margins. Medical records of the patients from whom the specimens were obtained were reviewed and analyzed for possible correlates to the status of specimen interpretability.
RESULTS: Histologic accuracy was sufficient in 46 cases (92%). Extensive heat distortion precluded full assessment of the ectocervical margins in ten (20%) and the endocervical margins in 22 (44%) of the cases. There was no difference in complete lesion evaluability whether LLETZ was performed solely for treatment in cases suitable for ablative procedures or for both diagnosis and treatment in patients who traditionally would have undergone a cone biopsy. If the latter group (N = 25) was analyzed separately, extensive heat distortion made histopathologic diagnosis impossible in four cases (16%) and precluded full assessment of the ectocervical margin in eight (32%) and the endocervical margin in 12 (48%).
CONCLUSION: The high rate of surgical-margin thermal destruction, with related limitation of interpretability, may represent a serious diagnostic and therapeutic limitation of the LLETZ procedure when considered as an alternative to cold knife conization.
PubMed ID: 8497366
Article Size: 2.5 MB
 

Telecytology: intraobserver and interobserver reproducibility in the diagnosis of cervical-vaginal smears.

Cervix, Other Anatomic Sites
Alli PM, Ollayos CW, Thompson LD, Kapadia I, Butler DR, Williams BH, Rosenthal DL, O’leary TJ.
Hum Pathol. 2001 Dec;32(12):1318-22.
Telecytologic diagnosis of cervical-vaginal smears is potentially useful because it could allow more efficient use of cytopathologist resources and expertise. A pathologist in one location could, in principle, review cytotechnologists’ findings using a video display hundreds or thousands of miles away. Currently, bandwidth restrictions limit practical implementation of such a system to review of fields that had been selected for review by the cytotechnologist. The purpose of our investigation was to evaluate how well this type of review correlates with a review in which the entire slide is available for examination by the pathologist. We prospectively selected 100 consecutive cervical-vaginal smears over an 11-day period in August 1999. For each smear, 4 to 12 fields containing abnormal cells from each slide were digitally imaged. Each of 3 pathologists reviewed all digitized images and all glass slides. Diagnoses based on selected digitized images were compared with those based on conventional pathologist review. The kappa statistic, a measure of chance-corrected agreement (reproducibility), was calculated in each setting. Overall, intraobserver and interobserver reproducibility of cervical-vaginal smear diagnoses is fair to excellent. The use of remote digital images for pathologist review did not introduce large (2-step) diagnostic disagreements. The disagreement between a pathologist’s glass slide and digital diagnoses is less than that for different pathologists reviewing glass slides, although interobserver differences were even greater in the interpretation of digital images.
PubMed ID: 11774163
Article Size: <1 MB
 

Primary osteosarcoma of the head and neck in pediatric patients: a clinicopathologic study of 22 cases with a review of the literature.

Bone, Other Anatomic Sites
Gadwal SR, Gannon FH, Fanburg-Smith JC, Becoskie EM, Thompson LD.
Cancer. 2001 Feb 1;91(3):598-605.
BACKGROUND: Primary osteosarcomas of the head and neck in the pediatric age group, not associated with previous irradiation or a known syndrome, are rare. The literature contains several single cases and small study series; however, to the authors’s knowledge, there has been no comprehensive large study to evaluate the clinicopathologic aspects of these tumors.
METHODS: Twenty-two cases of osteosarcomas of the head and neck in patients 18 years of age or younger, diagnosed between 1970 and 1997, were retrieved from the Otorhinolaryngic-Head & Neck Tumor Registry of the Armed Forces Institute of Pathology (AFIP). No secondary sarcomas (radiation-induced or those arising after chemotherapy) or those associated with known syndromes were included. Clinical, radiographic, and histologic features were reviewed, and patient follow-up was obtained.
RESULTS: The patients included 11 girls and 11 boys, 1-18 years of age (mean, 12.2 yrs). Patient symptoms related to tumor location were painless swelling, loss of teeth, headaches, or a mass lesion, present for an average of 5.9 months. No genetic abnormalities were documented. The tumors most frequently involved the mandible (n = 19), followed by the sphenoid sinus (n = 2) and the maxilla (n = 1). The tumors ranged in size from 1.1-10.0 cm (mean, 4.5 cm). All tumors were invasive and malignant by radiology and/or histology. The tumors were Grade 1 (n = 11), Grade 2 (n = 8), or Grade 3 (n = 3). All cases, except one chondroblastic osteosarcoma, were osteoblastic osteosarcomas. Thirteen patients underwent initial surgical resection with (n = 5) or without (n = 9) additional radiation and/or chemotherapy. The remaining 9 patients had an initial biopsy for diagnosis followed by surgery (n = 4) or surgery and radiation and/or chemotherapy (n = 5). Follow-up was available for 19 patients: 13 were alive at last follow-up with no evidence of disease (mean, 13.1 yrs); 1 was alive with disease (1.3 yrs); 3 had died without evidence of disease (mean, 23.2 yrs); and 2 had died of disease (mean, 7.8 yrs). The 3 patients with high-grade osteosarcoma were alive without disease (mean, 20.0 yrs).
CONCLUSIONS: Primary head and neck osteosarcomas in the pediatric population are typically low- to moderate-grade lesions in the mandible. Despite the invasive nature and high grade of a few of these tumors, there is an excellent overall long-term prognosis for patients in this age group with tumors in these locations.
PubMed ID: 11169944
Article Size: <1 MB
 

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

Hematopathology, Other Anatomic Sites
Neuhauser TS, Derringer GA, Thompson LD, Fanburg-Smith JC, Miettinen M, Saaristo A, Abbondanzo SL.
Mod Pathol. 2000 Sep;13(9):978-87.
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.
PubMed ID: 11007038
Article Size: 2 MB