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With an anatomical case and a clinical case, the authors describe the pygomelus, a very rare monstruosity. Legend and reality are often mixed in the description of literature. It is difficult to classify this monstruosity either in the simple monster or in the twin monsters.
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Hopeful monsters are organisms with a profound mutant phenotype that have the potential to establish a new evolutionary lineage. The Synthetic Theory of evolutionary biology has rejected the evolutionary relevance of hopeful monsters, but could not fully explain the mechanism and mode of macroevolution. On the other hand, several lines of evidence suggest that hopeful monsters played an important role during the origin of key innovations and novel body plans by saltational rather than gradual evolution. Homeotic mutants are identified as an especially promising class of hopeful monsters. Examples for animal and plant lineages that may have originated as hopeful monsters are given. Nevertheless, a brief review of the history of the concept of hopeful monsters reveals that it needs refinements and empirical tests if it is to be a useful addition to evolutionary biology. While evolutionary biology is traditionally zoocentric, hopeful monsters might be more relevant for plant than for animal evolution. Even though during recent years developmental genetics has provided detailed knowledge about how hopeful monsters can originate in the first place, we know almost nothing about their performance in natural populations and thus the ultimate difference between hopeful and hopeless. Studying the fitness of candidate hopeful monsters (suitable mutants with profound phenotype) in natural habitats thus remains a considerable challenge for the future.
A large number of duplication monstrosities have been observed in cattle, sheep, pigs, horses, goats, cats and dogs, ever since the publication of the famous woodcut of a swine double monster by J. S. Brant in Basel in 1496, better known as the "wunderbare Sau von Landser im Elsass". Albrecht Dürer also made a woodcut of this double monster in front of the village Landser in 1496. A picture of a deer double monster was published in 1603 by Heinrich Ulrich in Germany. In the monograph De monstrorum causis, natura et differentiis ..., published by the Italian Fortunius Licetus in 1616 pictures of double monsters being half man half dog are found. These fantasy figures have been popular for a long time and were supposed to be really in existence. Apart from these fantasy figures many pictures are known from real veterinary double monsters. U. Aldrovandus described in 1642 in his Monstrorum historia, besides many fantasy figures, also real human and veterinary double monsters and he gave also good pictures of them. In the 19th century examples of veterinary duplication monstrosities were published by I. Geoffroy Saint-Hilaire (1832-37), E. F. Gurlt (1832), W. Vrolik (1840) and C. Taruffi (1881); they proposed also concepts concerning the etiology. In the second volume of his famous handbook of teratology (1907), E. Schwalbe described many veterinary double monsters and discussed the theories of the genesis of congenital malformations. Various theories concerning the genesis of double monsters have been given since Aristotle (384-322 B.C.). ...
Using the metaphor of monsters, an analysis is made of the different ways in which the scientific community responds to uncertainties that are hard to tame. A monster is understood as a phenomenon that at the same moment fits into two categories that were considered to be mutually excluding, such as knowledge versus ignorance, objective versus subjective, facts versus values, prediction versus speculation, science versus policy. Four styles of coping with monsters in the science-policy interface can be distinguished with different degrees of tolerance towards the abnormal: monster-exorcism, monster-adaptation, monster-embracement, and monster-assimilation. Each of these responses can be observed in the learning process over the past decades and current practices of coping with uncertainties in the science policy interface on complex environmental problems. We might see this ongoing learning process of the scientific community of coping with complex systems as a dialectic process where one strategy tends to dominate the field until its limitations and shortcomings are recognized, followed by a rise of one of the other strategies. We now seem to find ourselves in a phase with growing focus on monster assimilation placing uncertainty at the heart of the science-policy and science-society interfaces.
We studied a life-saving method for the unaffected twin in two recent patients who had acardiac monster in twin pregnancy. We succeeded in blocking the umbilical blood flow of the acardiac monster in utero as radical treatment for this abnormality in one of the patients. In case 1, tocolysis and amniocentesis with puncture to the cyst of the acardiac monster were performed to prevent premature labor. However, the patient underwent premature labor at 27 weeks of gestation, and the newborn died. In case 2, fetal treatment was given: A steel coil was placed in the umbilical cord close to the abdominal wall of the acardiac monster under ultrasonographic guidance at 23 weeks of gestation to block blood flow. As a result, no enlargement of the acardiac monster was observed, and the cardiac function of the unaffected fetus improved. At 38 weeks of gestation, the patient delivered a normal baby weighing 2,237g and an acardiac monster weighing 110g. There were no complications in either the mother or newborn. There has been no report describing blockage of the umbilical blood flow of an acardiac monster in utero. Our method is considered efficient and less risky to the mother and the unaffected twin.
Nineteen cases of dermatofibroma associated with monster cells are reported. The term "monster" (an animal with a strange or terrifying shape, one unusually large for its kind) implies a strikingly atypical cell with an extremely large nucleus. Except for monster cells, these 19 lesions had all of the typical histopathological findings of dermatofibroma. The clinical diagnosis for 16 of these lesions was dermatofibroma (or histiocytoma). Three lesions were submitted without any clinical diagnosis. Eighteen of 19 lesions occurred on the extremities. One was on the back. Monster cells are seen in the early, histiocytic stage of dermatofibroma when lipophages and/or siderophages are usually present in large numbers. Only rarely were mitotic figures seen in dermatofibromas with monster cells, and they were neither numerous nor atypical. It is important for histopathologists to distinguish dermatofibroma with monster cells from cutaneous malignant fibrous histiocytoma and radiation sarcoma. The criteria for differentiation concern primarily the architectural pattern of the lesion rather than its cytological features.
Heteropolar doublets of the hypotrichous ciliate Oxytricha fallax can encyst, as can monster cells containing multiple copies of at least part of the ciliature. The resulting cysts excyst as heteropolar doublets or monster cells, respectively. Transmission electron microscopy of resting cysts derived from monster cells and from singlets, however, indicates a complete loss of the ciliature - all kinetosomes, ciliary axonemes, and microtubules. Thus, the determination of each ciliature of a monster cell cannot be attributed to the persistence of any visible part of the ciliature. Transmission electron microscopy of monster and singlet cysts suggests that the information for a ciliature may be related to the system of cyst wall grooves and possibly to differentiations in the inner cell membrane which separates the cyst cytoplasm from the cyst wall. The redevelopment of heteropolar doublets upon excystment indicates that the anterior-posterior polarity is independently determined for each ciliature and is maintained through cystment. This polarity is not, however, reflected in a recognizable anterior-posterior polarity of any visible structure of the resting cyst. The number of adoral zones of membranelles (AZMs) may increase or decrease in some excysting monster cells compared with that observed in the encysting cells. Possible explanations for these changes are offered in the Discussion.
Circulating nutrients serve as energy resources for functioning tissues throughout the body. While the tight regulation of plasma nutrients has been extensively studied in mammals, investigations into specific metabolic regulators in reptiles have been limited and have revealed conflicting results. The peptide exendin-4, which was isolated from the saliva of Gila monsters, Heloderma suspectum, has demonstrated prolonged plasma glucose-lowering properties in mammals. Although exendin-4 has often been labeled a venom protein, circulating plasma levels of exendin-4 have been shown to increase in response to feeding. Because exendin-4 has glucose-regulating effects in mammals, we hypothesized that post-prandial elevation in circulating exendin-4 levels in Gila monsters reduces plasma glucose and triglycerides. To examine the effect of exendin-4 on circulating nutrients, we measured plasma glucose, triglyceride, and cholesterol levels of Gila monsters in response to one of four treatments: fed live mice (a natural post-prandial increase in exendin-4), force-fed dead mice while anesthetized (no post-prandial exendin-4 increase), force-fed dead mice while anesthetized and injected with exendin-4 immediately after feeding (exogenous increase in exendin-4), and force-fed dead mice while anesthetized and injected with exendin-4 24 h after feeding (delayed exogenous increase in exendin-4). After prey ingestion, glucose and triglyceride levels increased significantly over time in all treatment groups, but there was no significant treatment effect. Plasma exendin-4 levels showed significant time and treatment effects, but did not correspond to glucose and triglyceride levels. Our results demonstrate that plasma nutrient levels in Gila monsters respond relatively slowly to feeding and that exendin-4 does not have the same effect on circulating glucose in Gila monsters as it does in mammals. Further studies are necessary to determine whether circulating exendin-4 has an alternate role in regulating other components of energy metabolism such as nutrient uptake rate in the small intestine.
Cells with significantly enlarged nuclei have been described in basal cell carcinomas, dermatofibromas, and pleomorphic fibromas, to name a few. These cells are typically visible using low power microscopy and have been termed "pleomorphic" or "monster cells." They have not been previously described in cutaneous melanomas. We sought to determine the prevalence of monster cells in otherwise conventional biopsies of primary cutaneous melanomas and its association with other histopathologic features of this malignancy. Ninety-nine superficial spreading melanomas, nodular melanomas, and acral lentiginous melanomas/lentigo malignas were retrospectively evaluated for the presence of monster cells, multinucleated giant cells, ulceration, inflammation, and depth of invasion (Breslow level). Thirteen cases of melanoma containing monster cells were found. A statistically significant association was noted between the presence of these cells, the histologic subtype of nodular melanoma (P = 0.0125), ulceration (P = 0.0127), the depth of invasion (P = 0.0103), and the presence of multinucleated giant cells (P = 0.0016). The finding of monster cells is not an uncommon occurrence and is seen more often in nodular melanomas.
Gila monster venom (1-300 micrograms/ml) is shown to inhibit completely the binding of [125I]VIP to human and rat intestinal epithelial cell membranes. In both models, the venom inhibits [125I]VIP binding and stimulates adenylate cyclase with a maximal efficiency that is similar to that of VIP and a potency that is 10000-50000 times lower than that of the peptide, on a weight basis. At maximal doses, VIP and Gila monster venom do not exert an additive effect on adenylate cyclase, suggesting that the activation of the enzyme by the venom occurs through VIP receptors. As is the case for VIP, adenylate cyclase activation by Gila monster venom requires the presence of GTP in the incubation medium. Finally, no VIP-like immunoreactivity was detected in the venom using an antiserum raised against mammalian VIP. All these data suggest the presence in the venom of the Gila monster, of a new substance which behaves as a VIP agonist in human as well as rat intestine.
We report a case of a 64-year-old woman with a giant dermatofibroma on her back with the unusual histologic feature of monster cells. The firm, exophytic, 3-cm nodule had purple and yellow components with surface telangiectasia. Histologic examination demonstrated characteristic findings of a dermatofibroma, including rete ridge flattening and bridging; a stroma containing scattered, large, round, eosinophilic collagen bundles; and a polymorphous dermal infiltrate of spindle and xanthomatous cells with scattered siderophages. Some xanthomatous cells demonstrated features consistent with monster cells, including huge bizarre nuclei and one or more nucleoli. Immunohistochemical staining for factor XIIIa was positive. A diagnosis of giant dermatofibroma with monster cells (DFMC) was made. Giant dermatofibromas are rare, with monster cells being an uncommon finding in dermatofibroma. To our knowledge, this is the first report of DFMC.
Venom from Gila monster (family Helodermatidae) contains a pancreatic secretagogue. In dispersed acini from guinea pig pancreas, the venom increased enzyme secretion to the same extent as did vasoactive intestinal peptide, secretin, or PHI. The abilities of vasoactive intestinal peptide and Gila monster venom to stimulate enzyme secretion were not altered by boiling but were abolished by incubation with trypsin or chymotrypsin. Like vasoactive intestinal peptide, secretin, and PHI, the venom caused a 50- to 60-fold increase in cellular cAMP and inhibited binding of 125I-vasoactive intestinal peptide to its membrane receptors on pancreatic acini. The action of venom on enzyme secretion was inhibited by [Gln9]secretin-(5-27), a vasoactive intestinal peptide receptor antagonist, but was not altered by atropine, a cholinergic receptor antagonist, or by dibutyryl cGMP, a cholecystokinin receptor antagonist. Gila monster venom contained no immunoreactive vasoactive intestinal peptide by radioimmunoassay. These results indicate that venom from Gila monster contains a peptide that can stimulate pancreatic enzyme secretion by interacting with vasoactive intestinal peptide receptors on pancreatic acinar cells and thereby activating adenylate cyclase and increasing cellular cAMP.
The Gila monster Heloderma suspectum is an active forager in an environment that, at times, can be extremely hot and arid. Thus, Gila monsters face extreme thermostatic and hydrostatic demands. For a desert ectotherm routinely risking dehydration, evaporative water loss (EWL) is typically viewed as detrimental. Yet evaporation simultaneously dehydrates and cools an animal. We explored EWL in Gila monsters by measuring cutaneous, ventilatory and cloacal EWL at five ambient temperatures between 20.5 degrees C and 40 degrees C. Our results show that Gila monsters have high EWL rates relative to body mass. Cutaneous EWL underwent a consistent, temperature-dependent increase over the entire range of test temperatures (Q(10)=1.61, with EWL ranging from 0.378 to 0.954 mg g(-1) h(-1)). Ventilatory EWL did not show a significant temperature-dependent response, but ranged from 0.304 to 0.663 mg g(-1) h(-1). Cloacal EWL was extremely low and relatively constant between 20.5 degrees C and 35 degrees C, but rose dramatically above 35 degrees C (Q(10) >8.3 x 10(7), from 0.0008 at 35 degrees C to 7.30 mg g(-1) h(-1) at 40 degrees C). This steep rise in cloacal EWL coincided with an increasing suppression of body temperature relative to ambient temperature. Dehydration to 80% of initial body mass led to a delay in the onset and an attenuation of the dramatic increase in cloacal EWL. These results emphasize the potential value of EWL for thermoregulation in ectotherms and demonstrate for the first time the role of the cloaca in this process.
The stimulatory effect of Gila monster venom on adenylate cyclase activity in rat pancreatic membranes was compared to that of porcine secretin and porcine VIP. The maximal effect exerted by the venom was identical to that of VIP but significantly lower than that of secretin. The effect of Gila monster venom could, however, be attributed to its interaction with secretin receptors rather than with VIP receptors, at variance with its previously described action on guinea pig pancreatic acini. Adenylate cyclase activation by both Gila monster venom and secretin in rat pancreatic membranes was, indeed: (1) dose-dependently inhibited by two secretin fragments secretin-(4-27) and secretin-(7-27), and (2) more severely depressed than VIP stimulation, after pretreating pancreatic membranes with dithiothreitol (DTT).