Frightened but lucid man who appears stated age. Vital signs are normal. No mass palpable in abdomen, though there is a faint suggestion of upper-abdominal distension. No supraclavicular lymphadenopathy, umbilical nodules, or upper-abdominal vascular bruit.
HISTORY
A 62-year-old man is admitted for chemotherapy for newly diagnosed pancreatic carcinoma. He has lost 8.2 kg (18 lb) in the past 9 weeks, although he has had only moderate anorexia.
PHYSICAL EXAMINATION
Frightened but lucid man who appears stated age. Vital signs are normal. No mass palpable in abdomen, though there is a faint suggestion of upper-abdominal distension. No supraclavicular lymphadenopathy, umbilical nodules, or upper-abdominal vascular bruit.
At once one notes marked hollowing of the temporal fossa, producing an impression of flesh loss far greater than what might be inferred, although the cheek is also visibly sunken (Figure). While such a change in contour is not pathognomonic of rapid unintentional weight loss, it strongly suggests cachexia, often of malignant origin.
The other immediate observation is of the sclera, which is anicteric. This patient had the misfortune that his cancer did not manifest itself earlier, as some ampullary pancreatic carcinomas do, by causing obstructive jaundice at a point when surgical cure is at least a consideration.
NATURE OF TEMPORAL FAT PAD AND MUSCLES
The temporal contour includes both the temporalis muscles that are familiar, and a temporal fat pad which is less so.1 The muscles are sharply attenuated as a result of generalized cachexia. This finding is particularly conspicuous in this instance because the muscle formed so distinctive a convex contour that its thinning stands out more sharply than, say, a hamstring or a single small muscle of the hand, to choose just 2 examples--though interosseous atrophy in the hand is perhaps the most common muscle atrophy that the generalist will see on physical examination in a large fraction of persons who are nutritionally compromised and in a great many aged persons.2 The on-edge view of the zygomatic arch is also especially striking in underscoring this sign (see Figure).
NUTRITIONAL COMPROMISE VERSUS OTHER CAUSES
Malnutrition in the absence of other causes--environmental starvation, that is--can produce this sign along with all the more unique and appalling features of kwashiorkor and marasmus; worldwide, this may be the leading source of temporal atrophy. Temporal atrophy in advanced HIV disease forms part of how AIDS, with or without superimposed mycobacterial infection, came to supplant isolated tuberculosis as the usual cause of "slim disease" in so much of the Third World and in the United States.3
Temporal hollowing can also develop in persons with sufficiently severe muscular degeneration in the absence of nutritional compromise or cachexia; this occurs in some myopathies and also after various surgical interruptions of the temporalis muscle fibers, their vasculature, or their innervation.1,4 When a systemic myopathy is the cause, this sign tends to be overwhelmed by functionally more pressing losses in the large muscles of locomotion and breathing, and the smaller ones of hand use, swallowing, and phonation.
There is also a constitutional variant in which slender but non-cachectic persons with none of the above problems exhibit sunken temples from childhood or adolescence onward. The descriptor "hatchet face" captures the impression created, that the face and head are narrowed in front and broader behind, but has too much potential for being construed as derogatory or demeaning to deserve widespread use; a better short term is needed.
PHYSICAL EXAMINATION IN PANCREATIC CANCER
Pancreatic cancer constituted an especially challenging diagnosis until the advent of CT and MRI, which now render the entire retroperitoneum accessible to imaging. Before that, indirect imaging signs were employed, such as pharmacologic dilation of the duodenum and inference from the status of the kidneys and ureters on intravenous pyelography. Physical examination is widely and aptly regarded as hopelessly inadequate in screening for or monitoring pancreatic neoplasms: the organ lies deep to "everything" in the abdomen and is backed by immense muscles that mean any displacement or access to inspection or palpation from the rear is but a pipe dream.
Nevertheless, several ingenious though insensitive physical signs have been described over time. These include the presence of a bruit originating in the splenic artery or vein as it courses over the superior surface of the pancreas.5 This occurs less often than it might because the lumen must be compromised to produce the turbulent flow associated with a bruit; in the absence of adhesions or engulfment in local spread, a splenic vessel pressed upon by an enlarged pancreas would merely be displaced. (At autopsy, the splenic artery and vein in middle-aged Americans are so commonly intensely tortuous that one wonders why we don't hear innocuous false-positive splenic-vascular bruits daily).
Another sign, albeit associated with incurability, is palpable induration of the navel from umbilical metastasis, the Sister Mary Joseph nodule--with almost any abdominal organ a potential source of the cancer.6,7 A "What's Your Diagnosis?" column on this topic will appear in the near future. Another equally nonspecific feature, and one indicative of distant lymph node metastasis, is the left supraclavicular mass of a Virchow node.8
Within the abdomen, the development of a palpable gallbladder with jaundice constitutes Courvoisier sign (not law9,10) and suggests that cancer or stricture obstructs the distal end of the biliary tree at or about the ampulla of Vater. Finally, the rare but dramatic abdominal extravasation signs seen in hemorrhagic pancreatitis and a variety of other conditions, namely the Cullen and Grey Turner signs,11-13 do not serve to mark pancreatic cancer.
Schneiderman H. Temporal atrophy in cachexia from pancreatic cancer. CONSULTANT. 2007;47:585-588.
REFERENCES:
1.
Kim S, Matic DB. The anatomy of temporal hollowing: the superficial temporal fat pad.
J Craniofac Surg.
2005;16:760-763.
2.
Schneiderman H, Ojeaburu J. Interosseous atrophy in neurodegenerative disease and ulnar palsy.
Consultant.
2000;40:2242-2246.
3.
Schneiderman H. "Slim disease" and AIDS-related cachexia.
Consultant.
1995; 35:379-382.
4.
Lacey M, Antonyshyn O. Use of porous high-density polyethylene implants in temporal contour reconstruction.
J Craniofac Surg.
1993;4:74-78.
5.
Serebro H. A diagnostic sign of carcinoma of the body of the pancreas.
Lancet.
1965;1:85-86.
6.
Powell FC, Cooper AJ, Massa MC, et al. Sister Mary Joseph's nodule: a clinical and histologic study.
J Am Acad Dermatol.
1984;10:610-615.
7.
Venu RP, Brown RD. Image of the month. Sister Mary Joseph's nodule: carcinoma of the pancreas with umbilical metastasis.
Gastroenterology.
1998;114: 632, 863.
8.
Taylor M, Gundling K, Schneiderman H. Right-sided "reverse Virchow's node" due to metastatic adenocarcinoma of unknown primary.
Consultant.
1994;34: 1293-1295.
9.
Parmar MS. Courvoisier's law.
CMAJ.
2003;168:876-877.
10.
Schneiderman H. It's not the law.
CMAJ.
2004;171:312.
11.
Chung MA, Oung CO, Szilagyi A. Cullen's sign: it doesn't always mean hemorrhagic pancreatitis.
Am J Gastroent.
1992;87:1026-1028.
12.
Schneiderman H, Singh NT. Atypically localized epigastric Cullen's sign.
Consultant.
1999;39:2296-2302.
13.
Guthrie C, Stanfey H. Rectus sheath haematoma presenting with Cullen's sign and Grey-Turner's sign.
Scott Med J.
1996;41:54-55.