Malignancies after hematopoietic cell transplantation for primary immune deficiencies: a report from the Center for International Blood and Marrow Transplant Research.

We describe the incidence of malignancy in patients with primary immunodeficiency disorders (PIDD) following hematopoietic cell transplantation (HCT). From the Center for International Blood and Marrow Transplant Research, 2266 PIDD patients who had undergone allogeneic HCT between 1968 and 2003 were identified. Patient, disease, and transplant factors for development of malignancy were examined and pathology reports for reported malignancies reviewed independently by a pathologist for confirmation. The incidence of malignancy was highest for Wiskott-Aldrich syndrome (3.3%), with an overall incidence of 2.3% for PIDD. Post-HCT malignancy was confirmed for 52 of 63 reported cases. Forty-five of 52 patients developed posttransplant lymphoproliferative disorders (PTLD) at a median of 3 months post-HCT. Of these, 26 had received T cell-depleted (TCD) bone marrow. Three patients who developed myelodysplastic syndrome had received TCD marrow and total body irradiation. Three patients developed a solid tumor. Patients with PIDD are at a relatively low risk of developing malignancies post-HCT compared with their historic risk of cancer. The most frequent malignancy or lymphoproliferative disorder was early-onset PTLD. As in other HCT recipients, TCD appears to correlate with PTLD development. Our results lend support to the hypothesis that immune reconstitution in PIDD following HCT leads to a decrease in cancer risk.

Laparoscopic splenectomy for metastatic squamous cell cancer of the neck.

Squamous cell carcinoma (SCC) spreads through direct extension, lymphatic vessels, and, rarely, hematogenous routes. The most common malignancies to metastasize to the spleen include carcinomas of the breast, lung, and melanoma. We present an unusual case of SCC of the neck with splenic metastases. The patient presented with a primary solid tumor of the neck that extended into the surrounding soft tissues, including the internal jugular vein and regional lymph nodes. A metastatic work-up with a positron emission tomograph showed enhancement in the left upper quadrant. A computed tomograph (CT) was then performed. The CT revealed three distinct splenic lesions, the largest measuring 6x6.5x2.5 cm. Subsequently, the patient was scheduled for a splenectomy. At the time of operation, diagnostic laparoscopy revealed only the splenic lesions. A laparoscopic splenectomy was performed successfully and the patient was started on a regular diet on postoperative day 1. This paper describes the first documented case of SCC of the neck with splenic metastases. The lesion was diagnosed and treated laparoscopically. The case is described in detail along with the clinical implications of this rare finding.

A unique presentation of a pharyngeal auricle.

Congenital lesions typically present in early childhood. Based on their location and presentation, the diagnosis and treatment is usually obvious. Because of the complexities and uniqueness of head and neck embryology, rare congenital lesions develop and this should be of interest to the otolaryngologist. In this report we present a rare case of a pharyngeal auricle that manifested itself as a polyp within the oropharynx of an infant.

Intralingual foregut duplication cyst in a newborn.

Congenital lingual cystic masses are challenging entities that can be detected prenatally or discovered in various forms after birth. We report what we believe is only the ninth case of an intralingual foregut duplication cyst that was lined with gastric mucosa. The preoperative evaluation of lingual cystic masses in newborns can include palpation, high-resolution sonography, computed tomography (CT), or magnetic resonance imaging (MRI). However, CT and MRI can pose a risk to the infant because they require sedation in order to properly position the patient for imaging. In our patient, we found that high-resolution sonography was most useful in revealing the nature of the mass as a nonvascular cystic lesion and in delineating its extension. We excised this lesion via bipolar cautery, which we prefer to cold-knife or CO2 laser surgery.

Safety and efficacy of intratesticular injection of vital dyes for lymphatic preservation during varicocelectomy.

PURPOSE: The most common complications of varicocelectomy include failure and hydrocele formation. Various surgical approaches have been used to minimize complications and guarantee durable results. The use of vital dyes to visualize and preserve testicular lymphatics intraoperatively has been proposed to reduce the incidence of hydrocele formation. We investigated the safety of intratesticular injection of various vital dyes and their efficacy in allowing visualization of lymphatics for varicocelectomy. MATERIALS AND METHODS: Using general anesthesia, 22 adolescent rats underwent bilateral transscrotal intratesticular injection of methylene blue, isosulfan blue, trypan blue or normal saline. All rats with intratesticular injection of a vital dye underwent immediate exploration of the spermatic cord to observe for visualization of lymphatic vessels. At 3 months all rats were euthanized, and orchiectomy was performed for histological examination. RESULTS: Spermatic cord lymphatic vessels were variably visualized depending on concentration and volume of the vital dye used. Histological examination of the testicles at 3 months after injection revealed areas with necrosis of seminiferous tubules, thickened tubular basement membranes, interstitial fibrosis and hyalinization, and striking intratubular dystrophic calcification. These pathological changes were present to varying degrees in all groups. CONCLUSIONS: Although spermatic cord lymphatic channels were successfully visualized to varying degrees, intratesticular injection of vital dyes (and even normal saline) produced pathological changes in all groups, despite variation of concentration, volume and type of vital dye. Intratesticular injection of vital dyes for the visualization of lymphatics during varicocelectomy should be abandoned. Paratesticular injection of vital dyes should be used with caution.

Primary cutaneous CD30-positive anaplastic large cell lymphoma in childhood: report of 4 cases and review of the literature.

We present the clinicopathologic findings in 4 children with primary cutaneous anaplastic large cell lymphoma (C-ALCL). The patients ranged in age from 13 months to 8 years, with 3 females and 1 male. All presented with a rapidly enlarging mass involving the skin and subcutaneous tissue. Histologic evaluation showed sheets of large pleomorphic lymphoid cells that were diffusely and strongly CD30(+). Tumor cells were CD45(+) in 1 of 4 cases. Cells were of T-cell phenotype, with variable positivity for CD3 (3 of 4 cases) and CD5 (2 of 4 cases). All 4 cases were positive for CD4 and clusterin. Staining for anaplastic lymphoma kinase was negative in all cases. No evidence of systemic involvement was noted at initial presentation or over a follow-up of 5 to 78 months, although 3 patients had cutaneous recurrences. Primary C-ALCL has only rarely been described in the pediatric population. The high-grade histologic appearance of this lymphoma belies its generally favorable clinical course and prognosis. Recognition of this entity and its differentiation from other T-cell lymphomas that secondarily involve the skin is important to avoid unnecessarily aggressive therapy in these children.

Plunging congenital epidermoid cyst of the oral cavity.

Epidermoid cysts are rare lesions in the oral cavity. They usually present early in life and are confined to one anatomical area. Simple excision is all that is needed. However, they may present late in life and be large in size, making their excision a real challenge. We present a case of congenital epidermoid cyst of the sublingual space that presented late, at the age of 17 years, after it plunged into the submental area. The presentation of the patient and the surgical approach are discussed.

Epstein-Barr virus-associated smooth muscle tumor of the basal ganglia in an HIV+ child: case report and review of the literature.

We describe the clinicopathologic features of an Epstein-Barr virus (EBV)-associated smooth muscle tumor arising in the basal ganglia of a 10-year-old human immunodeficiency virus (HIV)-positive child. Only a few cases of intracranial smooth muscle tumors are reported in the literature and virtually all of these have been extra-axial, involving the dura or sinuses in HIV+ adults. Our case underscores the need to include an EBV-associated smooth muscle tumor in the differential diagnosis when evaluating intracranial mass lesions in immunodeficient children.