A 40-year-old man presented with right upper lid swelling for several months. There was no pain or redness. He had received radiotherapy for nasopharyngeal carcinoma 12 years ago. The swelling did not respond to antibiotics nor non-steroidal anti-inflammatory medication. On examination, there was mechanical ptosis and loss of skin crease. The marginal reflex distance, palpebral fissure height (PFH), and levator function on the right eye were 2 mm, 8 mm, and 8 mm, respectively. Extraocular movement was normal and there was no proptosis (Fig 1a).

The diagnosis was periocular lymphoedema affecting mainly the right upper lid. He underwent right upper lid blepharoplasty, levator resection with biopsy, debulking of orbital septum, preseptal tissues and preaponeurotic fat pad, and lid crease formation under local anaesthesia. Histological examination revealed skin, adipose tissue and fibrovascular tissue with no evidence of malignancy. Postoperatively, there was significant improvement in symptoms and cosmesis (Fig 1b) and no recurrence after 9 months.

A 57-year-old woman underwent left hemiglossectomy and adjuvant radiotherapy for carcinoma of tongue. She presented with gradual swelling of the left upper lid causing visual obstruction. Examination revealed left upper lid swelling with secondary mechanical ptosis (Fig 2a). Her marginal reflex distance PFH, and levator function on the left eye were 0 mm, 4 mm, and 9 mm, respectively. There was also left hemifacial swelling. Visual acuity, intraocular pressure, fundal and pupil examination, and extraocular movement were all normal. There were no signs of recurrent carcinoma of tongue and no palpable cervical lymph nodes.

Similar to Case 1, surgical correction of eyelid lymphoedema was performed. Postoperatively, lid swelling resolved with reformation of lid crease (Fig 2b). The patient remained well 4 years after the surgery with no recurrence.

A 71-year-old man received radiotherapy for nasopharyngeal carcinoma in 1982. He presented with persistent non-pitting left upper lid swelling for 20 years. Incisional biopsy performed in 2004 revealed non-specific changes with no malignant cells. Additional eyelid surgery was offered but the patient opted for conservative management. Interval computed tomography imaging showed left eyelid swelling with no retrobulbar mass, and rectus muscles were not thickened. Examination revealed left upper lid swelling with secondary mechanical ptosis and loss of lid crease. Marginal reflex distance, PFH, and levator function on the left eye were 0 mm, 1 mm, and 5 mm, respectively. Extraocular movement and other physical examinations were normal.

Due to persistent symptoms, the patient elected surgical correction of eyelid lymphoedema. Biopsy showed no signs of malignancy. Postoperatively, left upper lid swelling resolved. The patient remained well 3 years postoperatively with no recurrence.

A 65-year-old woman presented with persistent right upper lid swelling in 2008. She had a history of nasopharyngeal carcinoma (T2N2M0) treated with radiotherapy in 1995. There was non-pitting oedema of the right upper lid. Liver and renal function tests were normal. She had no oedema in her extremities or elsewhere.

Computed tomography of the orbits showed smooth soft tissue oedema of the right upper lid with no abnormal mass. Cavernous sinuses were normal. Patchy sclerosis in the skull base was present and thought to represent post-radiotherapy changes.

Right upper lid blepharoplasty had been performed elsewhere in March 2009, where excessive and thickened skin was excised together with hypertrophic orbicularis muscles. The orbital septum was kept intact with preservation of the preaponeurotic fat pad. A similar repeat procedure was performed 4 months later by the same surgeon for persistent symptoms.

In 2019, the patient presented to us with residual bilateral upper lid oedema. Palpebral fissure height was 5 mm on the right eye and 6 mm on the left. Marginal reflex distance was 0 mm on the right eye and 1 mm on the left. Levator function was 9 mm on both eyes. There was secondary mechanical partial ptosis, loss of lid crease, and facial lymphoedema.

Surgical correction of eyelid lymphoedema was performed. Histological examination was compatible with lymphoedema. The patient recovered well postoperatively and there was no recurrence 6 months after surgery.

There are only a few reports of eyelid lymphoedema following neck dissection, surgery or radiation.1 2 3 As none of our cases underwent neck dissection, we hypothesise that radiotherapy alone can impede lymphatic drainage with consequent periocular lymphoedema. In this series, eyelid lymphoedema was frequently related to nasopharyngeal carcinoma, unlike other series.

Eyelid lymphoedema presents as a non-pitting oedema with thickening of the eyelid. In cases with severe swelling, visual field defect and disfigurement may occur.

Rosacea is the most reported eyelid lymphoedema association, albeit rare. More common presentations are blepharitis, conjunctivitis, and meibomianitis.3 4 5 6 7

Lymphoedema can usually be managed conservatively with manual drainage, compression garments, and skin care.8 Medical therapy with tetracycline and steroids have limited efficacy.4 5 7 Surgical treatment by debulking and split thickness skin grafting has been reported with favourable results.3 4 9 Lymphovenous anastomosis or bypass is also performed to divert lymphatic drainage to venous circulation.10 Nonetheless this is difficult in the periocular region where a lack of large superficial veins may result in an unsightly facial scar. Therefore, surgical debulking may remain the treatment of choice.

For surgical debulking, excessive skin and orbicularis should be addressed. Orbital septum debulking is essential since fluid tends to accumulate in this loose connective tissue. Debulking of the preaponeurotic fat pad can reduce upper lid swelling and enhance formation of skin crease. In our fourth case without such debulking, there was residual swelling. We addressed this in her last surgery resulting in marked improvement. In all four cases, there was no recurrence as the loose connective tissue was removed with orbital septum and preaponeurotic fat pad debulking. Levator resection should be performed in cases with long-standing oedema and levator muscle dysfunction. Skin crease forming sutures are placed for symmetry with the opposite eye, noting that Asian patients have thicker skin and orbicularis muscle, affecting skin crease formation and margin reflex distance. We prefer an incision 1 to 2 mm lower than the opposite normal side for better symmetry. Although surgery can improve eyelid oedema, patients should be informed that abnormal-appearing skin will persist. The longevity of improvement may be enhanced with presence of scar tissue making oedematous fluid less likely to accumulate.

Concept or design: All authors.
Acquisition of data: All authors.
Analysis or interpretation of data: All authors.
Drafting of the manuscript: All authors.
Critical revision of the manuscript for important intellectual content: HY Chan, HKL Yuen.

All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.

As an editor of the journal, HKL Yuen was not involved in the peer review process. Other authors have disclosed no conflicts of interest.

Case 2 in this study has been previously published in part in: Yuen KLH, Kwok YT. Surgical management of unusual eyelid swelling. iPlastics: official newsletter of the Asia Pacific Society of Ophthalmic Plastic and Reconstructive Surgery: 2016; 2(4): 4-6. Permission from the newsletter editor for publication of the case has been obtained. The authors confirm that there is no intentional or unintentional plagiarism in the present manuscript.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

This study was conducted in accordance with the Declaration of Helsinki. All patients were informed of the purpose of the study and their consent was obtained for all treatments, procedures, photography, and publication.

1. Sagili S, Selva D, Malhotra R. Eyelid lymphedema following neck dissection and radiotherapy. Ophthalmic Plast Reconstr Surg 2013;29:e146-9. Crossref

2. Possin ME, Burkat CN. Severe symmetric and chronic lower eyelid lymphedema in the setting of neck surgery and psoriasis. Open J Ophthalmol 2012;2:103-9. Crossref

3. Chalasani R, McNab A. Chronic lymphedema of the eyelid: case series. Orbit 2010;29:222-6. Crossref

4. Bernardini FP, Kersten RC, Khouri LM, Moin M, Kulwin DR, Mutasim DF. Chronic eyelid lymphedema and acne rosacea. Report of two cases. Ophthalmology 2000;107:2220-3. Crossref

5. Carruth BP, Meyer DR, Wladis EJ, et al. Extreme eyelid lymphedema associated with rosacea (Morbihan disease): case series, literature review, and therapeutic considerations. Ophthalmic Plast Reconstr Surg 2017;33(3S Suppl 1):S34-8. Crossref

6. Marzano A, Vezzoli P, Alessi E. Elephantoid oedema of the eyelids. J Eur Acad Dermatol Venereol 2004;18:459-62. Crossref

7. Lai TF, Leibovitch I, James C, Huilgol SC, Selva D. Rosacea lymphoedema of the eyelid. Acta Ophthalmol Scand 2004;82:765-7. Crossref

8. Mayrovitz HN. The standard of care for lymphedema: current concepts and physiological considerations. Lymphat Res Biol 2009;7:101-8. Crossref

9. Kabir SM, Raurell A, Ramakrishnan V. Lymphoedema of the eyelids. Br J Plast Surg 2002;55:153-4. Crossref

10. Chang EI, Skoracki RJ, Chang DW. Lymphovenous anastomosis bypass surgery. Semin Plast Surg 2018;32:22-7. Crossref

A 46-year-old lady with good past health presented to a tertiary hospital in August 2020 with a 6-month history of chronic cough, epigastric discomfort, and weight loss of 20 kg. She also reported progressive darkening and thickening of skin over both hands, neck, axilla, and groins since March 2020. She had consulted general practitioners and Chinese medicine practitioners and been given topicals and herbal treatment with no improvement. Physical examination revealed velvety hyperkeratosis and hyperpigmentation over both palms (Fig a), nape (Fig b), bilateral axilla (Fig c), and inguinal regions. The mucosal surfaces were not involved. Physical examination revealed stony dullness to percussion over the right mid-to-lower zone of the lungs and absent breath sounds on auscultation. A non-tender enlarged supraclavicular lymph node of 1 cm was palpable over the left supraclavicular region. Chest radiograph showed moderate right pleural effusion. Therapeutic thoracocentesis drained 2 L of clear straw-coloured fluid. Pleural fluid analysis revealed an exudative pleural effusion with 47.3 g/L fluid protein (70 g/L serum protein) and 224 U/L fluid lactate dehydrogenase (226 U/L serum lactate dehydrogenase). Pleural fluid cytology revealed suspicious cells with high nuclear-to-cytoplasmic ratio, coarse chromatin and enlarged, irregular hyperchromatic nuclei. Other laboratory findings were unremarkable: haemoglobin level 14 g/dL, white blood cell count 3.62 × 10⁹/L, platelet count 156 × 10⁹/L, creatinine level 71 μmol/L, bilirubin level 8 μmol/L, albumin level 28 g/L, alanine aminotransferase level 13 U/mL, and aspartate aminotransferase level 20 U/mL. Tumour markers including carcinoembryonic antigen, cancer antigen (CA) 15-3, CA 19-9, CA-125, and alpha-fetoprotein were all within the normal limits.

A clinical diagnosis of malignant acanthosis nigricans (AN) with tripe palms was made after dermatology review. Upper endoscopy revealed two Forrest class III gastric ulcers in the proximal greater curvature surrounded by abnormal 3-cm mucosal thickening with irregular mucosal surface and microvascular pattern under narrow-band imaging. Biopsy of the gastric ulcers was negative for Helicobacter pylori but confirmed poorly differentiated adenocarcinoma on histopathology. Positron emission tomography–computed tomography scan revealed a hypermetabolic focus in the stomach, multiple intra-abdominal lymph nodes, and a large hypermetabolic pelvic tumour. A clinical diagnosis was made of Krukenberg tumour. The patient was referred to medical oncology for palliative chemotherapy. She subsequently developed massive pulmonary embolism and succumbed 3 months after the initial diagnosis.

Acanthosis nigricans is a velvety hyperkeratotic, hyperpigmentation of the skin that occurs most commonly in intertriginous areas such as the back of the neck, axilla, and groins. Eight types of AN have been described and all share a common mechanism. They stimulate receptor tyrosine kinase signalling pathways; epidermal growth factor receptor (EGFR), insulin-like growth factor (IGF-1), and fibroblast growth factor receptors. Increased circulating insulin stimulates keratinocyte IGF receptors, especially IGF-1 and at high concentrations, displaces IGF-1 from IGF-1–binding protein. Increased serum-free IGF-1 in turn stimulates the proliferation of keratinocytes and dermal fibroblasts.1 2

Malignant AN is a paraneoplastic phenomenon most commonly associated with gastric adenocarcinoma with an incidence of 55% to 61%, followed by pancreatic cancer, gynaecological malignancies, and lung carcinoma.2 Increased transforming growth factor alpha (TGF-α) is postulated to be the underlying mechanism. The TGF-α acts on EGFR, stimulating soft tissue growth.1 Amelioration of malignant AN following tumour resection, associated with a reduction in elevated circulating TGF-α, supports the participation of EGFR signalling in malignant AN. Malignant AN can manifest preceding, together, or after diagnosis of an underlying malignancy. Rapid evolution of the velvety hyperpigmentation, tripe palms and signs of Leser-Trélat, a rare finding of sudden eruption of seborrhoeic keratoses, are strongly indicative of malignant AN.3 Affected patients are typically not obese and may be cachectic because of the underlying malignancies. Histological features are non-specific and commonly include hyperkeratosis, papillomatosis, basal layer hyperpigmentation, and some dermal papillae that project upwards in the form of finger-like projections.4 Malignant AN may resolve following tumour resection but can recur if there is tumour recurrence.

Krukenberg tumours are defined by the World Health Organization as ovarian carcinomas characterised by the presence of stromal involvement, mucin-producing neoplastic signet ring cells, and ovarian stromal sarcomatoid proliferation. The most common sites of primary malignancies are from the gastrointestinal tract and the breasts. The mean age at diagnosis of Krukenberg tumours is 49.3 ± 13.3 years. The prognosis is generally very poor, probably because of the late stage of diagnosis. The median survival time is 35.0 ± 3.5 months while the 5-year overall survival is around 25%.5

This case illustrates the classic presentation of malignant AN and tripe palms that are associated with metastatic gastric adenocarcinoma. Physicians need to be aware of these features since they may be the only presenting symptoms of the underlying malignancies. Full systemic evaluation for underlying malignancies is warranted to enable early diagnosis and timely management.

Concept or design: CPM Lam.
Acquisition of data: CPM Lam.
Analysis or interpretation of data: CPM Lam.
Drafting of the manuscript: CPM Lam.
Critical revision of the manuscript for important intellectual content: Both authors.

Both authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.

Both authors have disclosed no conflicts of interest.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

The patient was treated in accordance with the Declaration of Helsinki. Written informed consent for publication was obtained from the patient’s next-of-kin.

1. Phiske MM. An approach to acanthosis nigricans. Indian Dermatol Online J 2014;5:239-49. Crossref

2. DermNetNZ. Acanthosis nigricans. December 2021. Available from: https://dermnetnz.org/topics/acanthosis-nigricans. Accessed 18 Jul 2023.

3. Kilickap S, Yalcin B. Images in clinical medicine. The sign of Leser-Trélat. N Engl J Med 2007;356:2184. Crossref

4. Yu Q, Li XL, Ji G, et al. Malignant acanthosis nigricans: an early diagnostic clue for gastric adenocarcinoma. World J Surg Oncol 2017;15:208. Crossref

5. Xu KY, Gao H, Lian ZJ, Ding L, Li M, Gu J. Clinical analysis of Krukenberg tumours in patients with colorectal cancer—a review of 57 cases. World J Surg Oncol 2017;15:25. Crossref

A 60-year-old Chinese lady was admitted in August 2019 with fever, abdominal pain, and turbid peritoneal dialysate effluent. She had a history of end-stage renal disease due to immunoglobulin A nephropathy and had been on continuous ambulatory peritoneal dialysis for 3 years. Her usual medication included aspirin, calcitriol, cetirizine, ferrous sulphate, metoprolol, mirtazapine, pantoprazole, pregabalin, and sevelamer carbonate (1600 mg three times a day). Peritoneal dialysate fluid grew Escherichia coli, and intra-peritoneal gentamicin and ceftazidime were started. Her peritoneal dialysis catheter was removed 1 week later due to refractory peritonitis, but her abdominal pain persisted with development of paralytic ileus. A contrast computed tomography scan of abdomen performed 2 days following catheter removal revealed gross pneumoperitoneum and mural thickening over the small bowel. Laparotomy was performed and a proximal descending colonic ulcer with 2-mm perforation was identified. The patient underwent a left hemicolectomy but later succumbed in the intensive care unit due to hospital-acquired pneumonia.

An analysis of the colonic specimen demonstrated full thickness necrosis of the colonic wall with associated acute suppurative inflammation and peripheral ulceration. Incidentally there were abundant polygonal, non-refractile crystals with a brown, fish-scale configuration in the necrotic debris (Fig 1). The crystals appeared violet on periodic acid–Schiff stain staining. On haematoxylin and eosin staining, they had a two-toned colour imparted by pink linear accentuations (Fig 2).

Sevelamer is a calcium-free anion-exchange resin prescribed as a phosphate binder in patients with chronic kidney disease. It is composed of a non-absorbable hydrogel with ammonia on a carbon backbone. Stomach acid releases sevelamer polymer that binds phosphate in the intestine and forms a crystalline aggregate.1 Initially approved by the United States Food and Drug Administration in October 1998 as sevelamer hydrochloride, sevelamer has been largely replaced since 2007 by sevelamer carbonate.1 Sevelamer is commonly associated with gastrointestinal (GI) tract side-effects such as dyspepsia, abdominal pain, flatulence, and constipation.2 Sevelamer crystals (SCs) are non-polarised, have a broad curved and irregularly spaced fish-scale pattern, appear violet on periodic acid–Schiff staining, and have a two-tone yellowish/brownish colour on haematoxylin and eosin staining.1 About 19 cases of sevelamer-associated GI ulcers have been reported in the literature.3 The lesion can be found in all segments of the GI tract although the colon is the most common site. Sevelamer crystals are usually found inside the GI tract mucosa.2 Endoscopic findings include erosions and ulcerations, pseudo-inflammatory polyps and bezoar. Although a dose-dependent association had been reported, a recent review could not confirm the association.2 In one case report, a colonic mucosal ulcer developed while taking sevelamer carbonate 800 mg three times a day (duration not known).4 In another case report, a recto-sigmoid ulcer developed after taking sevelamer carbonate 1600 mg three times a day for 2 months.5 Diabetic patients appeared to be more prone to SC-associated GI lesions.2 They developed SC-associated GI lesions with a smaller dose compared with non-diabetics. Most reported cases required discontinuation of sevelamer,3 6 7 although improvement in clinical condition and cessation of rectal bleeding following dose reduction were reported in one case.2

In our patient, there were two possible explanations for her clinical course. She may have developed severe continuous ambulatory peritoneal dialysis peritonitis as a primary disease with secondary paralytic ileus, predisposing to SC deposition in the GI tract mucosa. Alternatively, she may have sustained GI tract injury by SC leading to colonic perforation and secondary peritonitis.

To the best of our knowledge, SC has not been previously reported to present with continuous ambulatory peritoneal dialysis peritonitis. The treating physician must be vigilant for potential complications of sevelamer prescribed in patients on peritoneal dialysis, especially when they have paralytic ileus.

Concept or design: YH Wong, SSH Wong.
Acquisition of data: YH Wong, SM Li.
Analysis or interpretation of data: YH Wong, SM Li.
Drafting of the manuscript: YH Wong, SM Li.
Critical revision of the manuscript for important intellectual content: All authors.

All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.

The authors have no conflicts of interest to disclose.

We thank Dr Ching-ki Fung and Dr Ngai-sheung Fung, pathologists of United Christian Hospital, for the detailed analysis of the colonic specimen and illustrative pathology images.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

The patient was treated in accordance with the Declaration of Helsinki. The patient’s next-of-kin has granted permission for submission and publication of this case report.

1. Swanson BJ, Limketkai BN, Liu TC, et al. Sevelamer crystals in the gastrointestinal tract (GIT): a new entity associated with mucosal injury. Am J Surg Pathol 2013;37:1686-93. Crossref

2. Yuste C, Mérida E, Hernández E, et al. Gastrointestinal complications induced by sevelamer crystals. Clin Kidney J 2017;10:539-44. Crossref

3. Uy PP, Vinsard DG, Hafeez S. Sevelamer-associated rectosigmoid ulcers in an end-stage renal disease patient. ACG Case Rep J 2018;5:e83. Crossref

4. Nambiar S, Pillai UK, Devasahayam J, Oliver T, Karippot A. Colonic mucosal ulceration and gastrointestinal bleeding associated with sevelamer crystal deposition in a patient with end stage renal disease. Case Rep Nephrol 2018;2018:4708068. Crossref

5. Tieu C, Moreira RK, Song LMWK, Majumder S, Papadakis KA, Hogan MC. A case report of sevelamer-associated recto-sigmoid ulcers. BMC Gastroenterol 2016;16:20. Crossref

6. Magee J, Robles M, Dunaway P. Sevelamer-induced gastrointestinal injury presenting as gastroenteritis. Case Rep Gastroenterol 2018;12:41-5. Crossref

7. Lai T, Frugoli A, Barrows B, Salehpour M. Sevelamer carbonate crystal–induced colitis. Case Rep Gastrointest Med 2020;2020:4646732. Crossref

A 71-year-old man, chronic smoker and drinker, presented to the Accident and Emergency Department of our institution in February 2021 with a history of abdominal pain and per rectal bleeding for 1 day. He also reported a weight loss of 15 kg over 1 month. On physical examination he was tachycardic and febrile; an abdominal mass was palpable over the right lower quadrant with localised peritoneal signs. Abdominal computed tomography revealed a 10 cm×9 cm×7.5 cm mass arising from the ascending colon with wall thickening of the caecum and ileum. There was also thickening of the perirenal fascia and a small amount of free fluid (Fig). Carcinoembryonic antigen (CEA) level was elevated (33 μg/L).

Laparotomy revealed an 8 cm×9 cm fungating tumour with circumferential involvement arising from the ascending colon. The tumour invaded the second and third portion of the duodenum, the right retroperitoneal space, ileocecal valve, and terminal ileum. It also presented with a concealed perforation sealed-off by the distal ileum without evidence of faecal contamination. There were no palpable liver masses and no signs of peritoneal deposits. Surgical excision of the tumour was performed to offer the best chance of survival. A right hemicolectomy with en bloc resection of the invaded structures was performed and a Roux-en-Y duodenojejunal anastomosis and end-to-end ileocolic anastomosis were fashioned.

The patient had a satisfactory postoperative recovery and was discharged from hospital under the care of our cancer care programme that included monitoring of CEA levels and annual colonoscopy and computed tomography of the abdomen and pelvis.

Interestingly, the histological examination revealed a carcinoma with squamous differentiation. Extensive sampling failed to reveal any glandular component. The final staging using TMN classification was Stage IIB (pT4bN0) and Dukes’ stage B. Due to the aggressive nature of the tumour, adjuvant chemotherapy was planned.

Soon after surgery, a lung mass was seen on chest X-ray and CEA level showed a rising trend. A positron emission tomography scan revealed multiple deposits over the abdominal cavity and a 2-cm right lung mass with mediastinal and right supraclavicular lymph node metastasis. An excisional biopsy of the supraclavicular lymph node was consistent with metastatic squamous cell carcinoma (SCC).

In view of the presence of multiple metastases the patient was commenced palliative chemotherapy for disease control with gemcitabine and carboplatin. Serial tomography also showed progression of the abdominal, lung and lymph node metastasis. His condition further deteriorated and he succumbed 7 months after the initial diagnosis.

Colorectal cancer (CRC) is the third most common cancer worldwide.1 In Hong Kong it is the second most common cancer and the second leading cause of cancer deaths.2

Most CRCs are adenocarcinomas and account for 95% of all cases. The remainder have non-epithelial histology such as carcinoid tumours, sarcomas, and lymphoid tumours. Squamous cell carcinoma accounts for only 0.1% to 0.5% of all types of CRC cases.3

The first case of SCC was reported in 1919 by Schmidtmann. The majority of the data available comes from individual case reports with only about 100 cases reported worldwide.3

The mean age at presentation is 55 to 60 years old with no gender or ethnic predilection. The most common sites are the rectum, right colon, and sigmoid. The clinical presentation is similar to that of colonic adenocarcinoma, such as altered bowel habit, rectal bleeding, abdominal pain, weight loss, anaemia, and palpable abdominal mass. The duration of symptoms ranges from several weeks to months. Lymphatic spread follows the same route as adenocarcinomas with similar metastatic sites such as the liver, peritoneum, lung, and bone.

Squamous cell carcinoma of the colon has been associated with ulcerative colitis, infection with human immunodeficiency virus, human papillomavirus, infestation with schistosomiasis, Entamoeba histolytica, history of previous surgical procedures, and radiotherapy.3 Nonetheless many reported cases have coexisting conditions.

The aetiology is unclear. There are three proposed pathogenic pathways, namely: (1) SCC arising from squamous differentiation from stem cells; (2) squamous metaplasia that undergoes malignant transformation; and (3) squamous differentiation from existing adenocarcinomas.4 The last pathway is supported by Williams et al4 who described squamous differentiation in three of 750 adenomas.

Miyamoto et al5 proposed a four-criteria selection for diagnosis: (1) metastasis from other sites must be excluded; (2) a squamous-lined fistulous tract must not involve the affected bowel; (3) SCC of the anus with proximal extension must be excluded; and (4) histological analysis must confirm the SCC.

Colorectal SCCs are more locally invasive and carry a worse prognosis than their common counterpart. Most cases are diagnosed at a late disease stage, often presenting as complications such as bowel obstruction or perforation. The overall 5-year survival of SCC of the colon is 35%, with 52% mortality within the first year, compared with the overall 60% 5-year survival of adenocarcinomas.3 Frizelle et al6 found that early stages of SCC had a similar prognosis to adenocarcinomas after evaluating 52 patients from the Mayo Clinic tissue registry in 2001. Nonetheless metastasis was present in 49% of these patients.

There is no current standard treatment. Most cases are managed following the guidelines for adenocarcinomas. The crucial steps are a complete surgical excision with negative margins, and aggressive chemotherapy. Various chemotherapy regimens have been proposed using 5-fluorouracil, capecitabine and gemcitabine.7 For SCC located in the rectum, chemoradiotherapy has demonstrated good success for local control, similar to anal SCC. The most important prognostic predictor is cancer stage. Factors associated with poor prognosis are a right-sided location and ulcerated or annular carcinomas.

Considering only 10% to 20% of all CRC cases present with local invasion, this feature should alert surgeons to this form of aggressive CRC. The timing of post-treatment surveillance (serial CEA and annual tomography and colonoscopy) can be adjusted considering the higher mortality and worse prognosis. Systemic staging investigations such as computed tomography thorax or positron emission tomography scan can be regularly implemented in view of the higher rate of metastasis.

Both authors contributed to the concept or design of the study, acquisition of data, analysis or interpretation of data, drafting of the manuscript, and critical revision of the manuscript for important intellectual content. Both authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.

Both authors have no conflicts of interest to disclose.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

The patient was treated in accordance with the Declaration of Helsinki and provided informed consent for the treatment/procedures and verbal consent for publication.

1. World Health Organization. Colorectal cancer. 2020. Available from: https://www.iarc.who.int/cancer-type/colorectal-cancer/. Accessed 5 Jun 2023.

2. Hong Kong Cancer Registry. Top ten cancers. 2020. Available from: https://www3.ha.org.hk/cancereg/topten.html. Accessed 5 Jun 2023.

3. Linardoutsos D, Frountzas M, Feakins RM, Patel NH, Simanskaite V, Patel H. Primary colonic squamous cell carcinoma: a case report and review of the literature. Ann R Coll Surg Engl 2020;102:e1-7. Crossref

4. Williams GT, Blackshaw AJ, Morson BC. Squamous carcinoma of the colorectum and its genesis. J Pathol 1979;129:139-47. Crossref

5. Miyamoto H, Nishioka M, Kurita N, et al. Squamous cell carcinoma of the descending colon: report of a case and literature review. Case Rep Gastroenterol 2007;1:77-83. Crossref

6. Frizelle FA, Hobday KS, Batts KP, Nelson H. Adenosquamous and squamous carcinoma of the colon and upper rectum: a clinical and histopathologic study. Dis Colon Rectum 2001;44:341-6. Crossref

7. Wang ML, Heriot A, Leong T, Ngan SY. Chemoradiotherapy in the management of primary squamous-cell carcinoma of the rectum. Colorectal Dis 2011;13:296-301. Crossref

A 27-year-old female was admitted in January 2021 to the cardiovascular department of Ege University, Turkey with a history of a gradually enlarging swelling in the right supraclavicular area. Physical examination revealed a semi-soft, semi-mobile and painless mass localised at the bottom third of the right lower neck, extending to the clavicle with no distinct inferior border. It appeared to follow upper mediastinal inflow. The overlying skin was normal, and no pulsation or thrill was detected. All laboratory results including infection markers were within normal limits. Sonographic evaluation demonstrated a solid, hypoechoic and lobulated mass with slow flow pattern and slow filling of the right internal jugular vein (IJV). Computed tomography scan illustrated a homogenous oval solid lesion on the right lateral aspect of the neck, originating from the thyroid level and elongating to the infraclavicular area. It measured 33 × 51 × 51 mm, with peripheral interrupted nodules in arterial phase (Fig 1a). The mass was located 180° to the IJV. The border could not be differentiated and the trachea was deviated medially to the left and right common carotid artery posteriorly. Magnetic resonance imaging demonstrated intermediate signal intensity on T1-weighted image (Fig 1b) and very high signal intensity on T2-weighted image of an enhanced solid mass (Fig 1c). The clinical diagnosis was vascular malformation or haemangioma. Surgical excision was planned. After general anaesthesia and positioning of the neck, a neck incision was made parallel and anterior to the right sternocleidomastoid muscle, similar to that performed for a standard carotid endarterectomy. Although the incision was extended through the sternal notch, it was insufficient to enable complete excision. A right-sided mini ‘J-sternotomy’ was performed subsequently to facilitate complete visualisation of the brachiocephalic bifurcation. Exploration and dissection of the mass from peripheral tissue revealed that it arose from the distal part of the right IJV and extended through the brachiocephalic bifurcation.

The proximal part of the right IJV, right subclavian vein and distal part of the right innominate vein were explored and controlled by silicon loop. Following heparin administration, the proximal right subclavian vein was ligated and all other vessels clamped. An incision was made and the mass was observed to extend into the vascular lumen. It was removed en bloc along with the distal segment of the right IJV and proximal segment of the right brachiocephalic vein. Vein reconstruction was performed to prevent venous hypertension in the neck. Because of the large diameter of vascular structures, a synthetic 8-mm polytetrafluoroethylene self-ringed graft was sutured between the distal part of the right innominate bifurcation and right IJV (Fig 2a and 2b). Histopathology revealed CD34(+), endothelial cell(+) haemangioma. The patient experienced no postoperative complications. Both antiaggregant and anticoagulant therapy were commenced on postoperative day 1 with acetylsalicylic acid (100 mg/day) and apiksaban (5 mg/day). Control computed tomography scan 11 months after surgery revealed excellent reconstruction with an intact and patent graft (Fig 2c).

The nomenclature of primary tumours of the venous system is based on their origin: lipomas, leiomyomas, haemangiomas, leiomyosarcomas, and angiosarcomas. Leiomyosarcomas are the most common tumours with a malignant course. Until the classification scheme proposed by Mulliken and Glowacki,1 the terms ‘haemangioma’ and ‘vascular malformation’ were used interchangeably because of the lack of a standardised nomenclature. Although they are classified as benign tumours, accurate diagnosis is possible only by histopathological evaluation following surgical excision. According to their classification, haemangiomas are characterised by rapidly proliferating endothelial cells and frequent mitosis. They are not usually visible at birth but become apparent at the neonatal stage and demonstrate rapid proliferation during the first 2 years, followed by spontaneous involution. In contrast, vascular malformations possess flattened endothelial cells and ectatic vessel formation. Although haemangiomas grow with hyperplasia, vascular malformations expand by hypertrophy.1 Some authors have noted that trauma, sepsis, hormonal changes or pressure in the venous system may cause expansion of the vascular malformation.2 Although cases of external jugular vein haemangiomas and vascular malformations have been reported,2 only three cases of IJV haemangioma have been reported. The exceptions were incidentally diagnosed asymptomatic cases; these presented with swelling and may have led to incorrect differential diagnoses of neck malignancy, infection, lymphoma or thrombosis. A multidisciplinary approach with a full physical examination and medical history is required to reach a definitive diagnosis. Advanced imaging techniques play an important role in diagnosis and are helpful when planning treatment. The sonographic and magnetic resonance imaging features of vascular malformations and haemangiomas have been studied and described in detail. In a case series, Ahuja et al3 reported the radiological features of vascular malformations in the external jugular vein, aiming to help clinicians make treatment decisions. Head and neck vascular malformations typically showed intermediate signal intensity on T1-weighted images, very high signal intensity on T2-weighted images and variable enhancement following intravenous administration of gadolinium.4 Similar radiological features were identified in our case. To date, different treatment modalities such as cryotherapy, laser therapy, and steroids have been explored. Excision of a benign vein tumour with the adjacent vein segment en bloc is the most successful curative treatment and enables pathological confirmation of the diagnosis.5

In previous case studies of IJV haemangiomas, a supraclavicular approach was applied to access the mass and reconstruction not performed following removal of the vein segment.5 In our challenging case, the neck incision was extended to the sternal notch and continued with a right-sided mini ‘J-sternotomy’ to ensure adequate control over the mass. Contrary to other case reports of IJV haemangioma, we performed vein reconstruction to prevent venous hypertension and swelling of the neck. To the best of our knowledge, this is the first reported case where vein reconstruction was performed using a synthetic graft following successful surgical excision of a haemangioma from the IJV via an extraordinary approach.

Concept or design: Ö Balcıoğlu, S Ertugay. Acquisition of data: S Ertugay, H Posacıoğlu. Analysis or interpretation of data: All authors. Drafting of the manuscript: Ö Balcıoğlu, S Ertugay. Critical revision of the manuscript for important intellectual content: All authors.

All authors had full access to the data, contributed to the study, approved the final version for publication, and take responsibility for its accuracy and integrity.

All authors have disclosed no conflicts of interest.

This study received no specific grant from any funding agency in the public, commercial, or not-for-profit sectors.

The patient was treated in accordance with the Declaration of Helsinki and provided written informed consent for publication of this report.

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2. de Oliveira JC, Barreto FT, Chimelli BC, et al. External jugular vein hemangioma: case report. J Vasc Bras 2019;18:e20180026. Crossref

3. Ahuja AT, Yuen HY, Wong KT, et al. External jugular vein vascular malformation: sonographic and MR imaging appearances. AJNR Am J Neuroradiol 2004;25:338-42.

4. Werner JA, Dünne AA, Folz BJ, et al. Current concepts in the classification, diagnosis and treatment of hemangiomas and vascular malformations of the head and neck. Eur Arch Otorhinolaryngol 2001;258:141-9. Crossref

5. Duggal P, Chaturvedi P, Pai PS, Nair D, Juvekar SL, Rekhi B. Internal jugular vein vascular malformation presenting as mass at root of neck: a case report. BMC Ear Nose Throat Disord 2009;9:5. Crossref