Rare Case of Leiomyoma and Adenomyosis in Mayer-Rokitansky-Kuster-Hauser Syndrome.

We report a case of adenomyosis which developed from a hypoplastic uterus and leiomyoma in a patient with MRKH syndrome. A 45-year-old Malay female with primary amenorrhoea and primary infertility presented with abdominal mass and abdominal pain. She is phenotypically female, has well developed secondary sexual characteristics, and has normal female external genitalia with shallow vagina dimple. Transabdominal ultrasonography showed a homogenous adnexal mass of 10 × 8 cm, uterus sized 5 × 4 cm, and normal kidneys. A complex mass of right adnexa was demonstrated by CT scan. Exploratory laparotomy showed torsion of right adnexal mass and rudimentary uterus with fibroid but no endometrial tissue and blind end with absent cervix. The normal right ovary and tube were not visualized. The left fallopian tube and ovary were normal. It is also complicated by vaginal agenesis. Removal of right adnexal mass and rudimentary uterus was done with preservation of left ovary. The histologic diagnosis was uterine adenomyosis and leiomyoma arising from the right adnexa, possibly from the broad ligament.

Peripheral, but not central, axotomy induces neuropilin-1 mRNA expression in adult large diameter primary sensory neurons.

Neuropilin-1 (NP-1) is a component of the receptor for semaphorin3a (Sema3a), a member of a large family of molecules with widespread expression and demonstrable influence (via their ability to repel growing axons) on nervous system development. Recent studies have shown that some types of adult mammalian neurons retain the capacity to respond to Sema3a, particularly in relation to neuronal injury and regeneration. Although variations in expression of Sema3a mRNA have been revealed in neurons in both the central and peripheral nervous systems in this context, relatively little is known about NP-1 expression patterns. In this study we investigated the expression of NP-1 mRNA in adult dorsal root ganglion (DRG) neurons in intact and lesioned animals. We compared the effect of unilateral lesioning of the sciatic nerve or unilateral dorsal rhizotomy at lumbar levels L4/5, and bilateral dorsal funiculus lesioning at thoracic levels T10/11 on NP-1 mRNA expression in the cell bodies of lumbar DRGs. A significantly increased level of NP-1 mRNA expression was detected only following sciatic nerve lesioning (P < 0.001), but not after rhizotomy or dorsal funiculus lesioning. Furthermore, this upregulation was mainly confined to large diameter neurons of DRGs at lumbar levels L4/5, which provide the main sensory contribution to the sciatic nerve. These results suggest a role for NP-1 in the axonal response to peripheral nerve injury, which may be specific to a particular subset of primary sensory neurons.

Neuropilin-1 is expressed on adult mammalian dorsal root ganglion neurons and mediates semaphorin3a/collapsin-1-induced growth cone collapse by small diameter sensory afferents.

Neuropilin-1 on the growth cones of NGF-dependent embryonic dorsal root ganglion (DRG) neurons mediates the repulsive effects of secreted semaphorin3a, but its role in adult neurons is unknown. Here we show that most adult rat DRG neurons, regardless of cell diameter/afferent phenotype, express neuropilin-1 protein in vitro. However, the response of growth cones belonging to these neurons (induced by recombinant collapsin-1/semaphorin3a and blocked by the anti-neuropilin-1 antibody) was restricted to those of small cell body diameter (<30 microm), corresponding primarily to nociceptive sensory afferents. Neurotrophic factors had a differential effect on neuropilin-1 expression in vitro, with DRG neurons cultured in either NGF or GDNF expressing the highest levels on their neurites. These findings suggest that neuropilin-1-mediated repellent effects of semaphorins may regulate the behavior of nociceptive sensory axons in the adult as well as the embryonic peripheral nervous system.

Differential expression of adhesion molecules within the human thymus.

Development of a diverse, MHC-restricted yet self-tolerant T-cell repertoire occurs within the thymus, and requires contact between developing T cells and their stromal microenvironment. Such interactions are likely to depend on the combinatorial effect of specific adhesion molecules. As a preliminary step to determining their role in T-cell development, we have studied the distribution of LFA-1/ICAM-1, CD2/LFA-3, VLA-4/VCAM-1, and HECA 452-antigen/E-Selectin ligand pairs on frozen sections of human thymus. Using two color-immunohistochemistry, and a variety of cell-lineage markers that reveal the nature of the cells on which these adhesion molecules are located, we find a differential distribution of adhesion molecules, with some being shared by both endothelial and epithelial cells. We also identify the VCAM-1-positive subpopulation as cortical macrophages. The relevance of these findings to thymopoiesis is discussed.